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January 2018 Novel Role of Deacetylase 11 (HDAC11) in Regulating Normal and Malignant Hematopoiesis Jie Chen University of South Florida, [email protected]

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Scholar Commons Citation Chen, Jie, "Novel Role of 11 (HDAC11) in Regulating Normal and Malignant Hematopoiesis" (2018). Graduate Theses and Dissertations. https://scholarcommons.usf.edu/etd/7608

This Dissertation is brought to you for free and open access by the Graduate School at Scholar Commons. It has been accepted for inclusion in Graduate Theses and Dissertations by an authorized administrator of Scholar Commons. For more information, please contact [email protected]. Novel Role of Histone Deacetylase 11 (HDAC11) in Regulating Normal and

Malignant Hematopoiesis

by

Jie Chen

A dissertation submitted in partial fulfillment of the requirements for the degree of Doctor of Philosophy Department of Cell Biology, Microbiology and Molecular Biology College of Arts and Sciences University of South Florida

Major Professor: Eduardo M. Sotomayor, M.D. Co-Major Professor: Javier Pinilla Ibarz, M.D., Ph.D. Sheng Wei, M.D. Kenneth Wright, Ph.D.

Date of Approval: Dec 21, 2017

Keywords: Neutrophils, Myeloid-derived suppressor cells, CD8+ T cells, C/EBPbeta,

Copyright © 2017, Jie Chen

Table of Contents

List of Figures ...... v

Abstract ...... vii

Chapter 1: Background ...... 1 1.1 Cancer ...... 1 1.1.1 Cancer and host immune cyctem...... 2 1.1.2 Hematopoiesis and abnormal myelopoiesis ...... 4 1.1.3 Current approaches for cancer immunotherapy ...... 6 1.2 Tumor microenvironment ...... 7 1.2.1 Tumor infiltrated T lymphocytes ...... 8 1.2.2 Tumor-derived suppressor cells ...... 9 1.2.2.1 The origin of MDSCs ...... 10 1.2.2.2 MDSCs expansion ...... 11 1.2.2.3 Subsets of MDSCs ...... 11 1.2.2.4 Mechanisms regulating MDSCs biology ...... 12 1.2.2.5 Clinical evaluation of MDSCs ...... 15 1.2.2.6 Targeting MDSCs in Cancer ...... 16 1.2.3 Tumor associated macrophages ...... 17 1.2.4 Other cellular and non-cellular components in the tumor microenvironment ...... 18 1.2.5 Strategies of targeting the tumor microenvironment ...... 19 1.3 Histone deacetylases ...... 22

i 1.3.1 Classification of HDACs ...... 22 1.3.2 HDAC11 ...... 23 1.3.2.1 HDAC11 in APCs ...... 25 1.3.2.2 HDAC11 in T lymphocytes ...... 26 1.3.2.3 HDAC11 in cancer ...... 27 1.3.2.4 Other functions of HDAC11 ...... 27 1.3.3 Other HDACs ...... 28 1.3.4 HDAC inhibitors ...... 29

Chapter 2: Essential Role for Histone Deacetylase 11 (HDAC11) in Neutrophil Biology ...... 33 2.1 List of abbreviation ...... 33 2.2 Abstract ...... 35 2.3 Introduction ...... 36 2.4 Material and methods ...... 39 2.4.1 Mice and cell lines ...... 39 2.4.2 Quantitative reverse transcriptase-polymerase chain reaction (qRT- PCR) ...... 39 2.4.3 Flow cytometry ...... 40 2.4.4 Immunoblotting ...... 41 2.4.5 Harvesting and in vitro culturing of mouse peritoneal neutrophils ... 41 2.4.6 Migration assay analysis ...... 42 2.4.7 Chromatin immunoprecipitation ...... 42 2.4.8 Phagocytosis assay ...... 43 2.4.9 Incucyte real-time microscopy analysis ...... 44 2.4.10 Cytometric bead array ...... 44 2.4.11 Sepsis model ...... 45 2.4.12 RACE analysis ...... 45

ii 2.4.13 Statistical Analysis ...... 45 2.5 Results...... 45 2.5.1 HDAC11 is differentially expressed in various stages of myeloid differentiation...... 45 2.5.2 Expression of HDAC11 is significantly increased in leukemic cells upon differentiation ...... 48 2.5.3 Lack of HDAC11 markedly increases the expression of pro- inflammatory cytokines ...... 49 2.5.4 Neutrophils isolated from mice lacking HDAC11 demonstrate much higher migratory and phagocytic capacity ...... 53 2.5.5 HDAC11KO mice are more susceptible to LPS induced sepsis when compared to C57BL/6 wild-type counterpart ...... 56 2.5.6 HDAC11KO mice show bone marrow hypercellularity with granulocytic expansion and splenomegaly due to increased extramedullay hematopoiesis ...... 58 2.6 Discussion ...... 61

Chapter 3: HDAC11 Function as a Transcriptional Regulator in Immature Myeloid Cells (IMCs) to Myeloid-derived Suppressor Cells (MDSCs) transition ...... 67 3.1 List of abbreviation ...... 67 3.2 Abstract ...... 68 3.3 Introduction ...... 69 3.4 Material and methods ...... 72 3.4.1 Mice and cell line ...... 72 3.4.2 Genotyping ...... 73 3.4.3 In Vivo tumor model ...... 74 3.4.4 Splenic and tumor infiltrated MDSCs isolation ...... 74 3.4.5 BM-derived MDSCs generation ...... 75 3.4.6 activity assay ...... 75 3.4.7 NO production assay ...... 76 3.4.8 ROS production ...... 76

iii 3.4.9 Quantitative real-time pCR (qRT-PCR) ...... 77 3.4.10 Flow cytometry analysis and cell sorting ...... 77 3.4.11 Western blotting analysis ...... 78 3.4.12 Adoptive Cell Therapy ...... 78 3.4.13 Chromatin immunoprecipitation (ChIP) ...... 79 3.4.14 Statistics ...... 80 3.5 Results...... 80 3.5.1 C/EBPβ is necessary for bone marrow-derived and tumor-induce MDSCs ...... 80 3.5.2 Elevated level of C/EBPβ contributes to the up-regulation of involved with MDSC suppression in HDAC11KO mice .. 83 3.5.3 HDAC11 deletion promotes MDSCs suppressive activity through increased arginase activity and NO production ...... 85 3.5.4 HDAC11 negatively regulates C/EBPβ expression by interacting with the promoter region of this transcription factor ...... 89 3.5.5 Mice with HDAC11 deletion in myeloid compartment displayed enhanced tumor progression compared to total knockout mice .... 90 3.5.6 HDAC11 plays a contradictory role in regulating MDSCs and T cells in the tumor microenvironment ...... 92 3.6 Discussion ...... 94

Chapter 4: Conclusion and Future Directions ...... 99

List of references ...... 104

Appendices ...... 114

iv

List of Figures

Figure 2.1 HDAC11 message is differentially expressed in various stages of myelopoiesis ...... 47

Figure 2.2 Expression of HDAC11 in acute promyelocytic leukemia samples (APL) and functional consequences of its manipulation in this model ...... 49

Figure 2.3 ...... 51

Figure 2.4 Phenotypic consequences of HDAC11 deficient peritoneal neutrophils ..... 53

Figure 2.5 Enhanced migratory and phagocytic capacity of HDAC11KO neutrophils . 55

Figure 2.6 Septic shock experiment comparing HDAC11KO mice and C56BL/6 mice 58

Figure 2.7 Observation of splenomegaly as well as Hypercellularity with granulocytic expansion in BM of HDAC11KO mice ...... 60

Figure 2.8 ...... 65

Figure 3.1 C/EBPβ expression is up-regulated in MDSCs with HDAC11 deletion ...... 82

Figure 3.2 C/EBPβ is involved with the suppressive activity of MDSCs lacking HDAC11 ...... 84

v Figure 3.3 Enzymes involved in the suppressive pathways employed by BM-derived MDSCs with HDAC11 deletion ...... 86

Figure 3.4 Enzymes involved in the suppressive pathways employed by HDAC11KO MDSCs in vivo ...... 88

Figure 3.5 HDAC 11 regulates C/EBPβ expression in MDSCs ...... 90

Figure 3.6 Mice lacking HDAC11 in the myeloid compartment demonstrate a more enhanced tumor growth pattern than HDAC11 total knockout ...... 91

Figure 3.7 The antitumor efficacy by adoptive transfer of HDAC11KO T cells can be diminished by MDSCs lacking HDAC11 ...... 93

vi

Abstract

During hematopoiesis, multilineage progenitor cells and the precursors are

committed to individual hematopoietic lineages. In normal myelopoiesis, the immature

myeloid cells (IMCs) differentiate into macrophages, neutrophils or dendritic cells.

However, under tumor burden, these IMCs differentiate into myeloid derived suppressor

cells (MDSCs) result in an up-regulation of immune suppressive factors and pro-tumor

effect. The development of normal or malignant is tightly controlled by endogenous

signals such as transcription factors and epigenetic regulations. HDAC11 is the newest

identified members of the histone deacetylase (HDAC) family. Previous study in our

group had identified HDAC11 as a negative regulator of interleukin 10 (IL-10) production

in antigen-presenting cells (APCs). However, the mechanisms of HDAC11 in regulating

myeloid cells differentiation and function remained unclear.

We have uncovered for the first time that in the absence of HDAC11, upon LPS

stimulation, neutrophils isolated form mice displays an over-production of pro-

inflammatory cytokines such as TNF-alpha and IL-6. Strikingly, these HDAC11KO neutrophils showed a significantly higher migratory and phagocytosis activity, resulting from an overexpression of the migratory receptor and cytokine CXCR/L2. We have performed Chromatin Immunoprecipitation (ChIP) analysis on the neutrophils and discovered that HDAC11 was recruited to the promoter regulatory region of these we have identified. This part of data will be discussed mainly in chapter 2.

vii Not only does HDAC11 plays a crucial role in the neutrophil function, our group have also found out that lacking of HDAC11 result in an increased suppressive activity of the Myeloid-derived Suppressor Cells (MDSCs). The previous publication of our group had shown that the tumor bearing mice experienced a much more aggressive growth pattern in the HDAC11 KO mice compare with C57BL/6 wild type control.

MDSCs isolated from mice lacking HDAC11 appeared to gain increased capability to suppress the function of antigen-specific CD8+ T cells in vitro. Followed by this initial study, in chapter 3, we observed an up-regulation of both expression and enzymatic activity of arginase 1 and Nos2, two enzymes that are crucial in regulating MDSCs suppressive function. The aberrant enzymatic activity of Arg1 and Nos2 in HDAC11KO

MDSCs is possibly result from an over-expression of the lineage-specific transcription factor C/EBPβ, which is previously proved to be essential for the differentiation of functional MDSCs. Furthermore, our ChIP data confirmed that HDAC11 may play as an negative regulator of C/EBPβ. Recently, our lab had demonstrated that T cells lacking

HDAC11 gained a hyperactive phenotype and anti-tumor effect, indicating that HDAC11 may play a dual role in the host immune system. We further performed an adoptive transfer therapy to C57BL/6 tumor bearing mice. Our data showed that the additional administration of HDAC11KO MDSCs could eliminate, at least partially, the anti-tumor effect by adoptive transfer of HDAC11KO T cells.

Taken together, we have uncovered a previously unknown role for HDAC11 as a transcriptional regulator in the myeloid cells differentiation and function. Based on our data and previous work from our lab, we propose a dual role of HDAC11 played in the host immune system. In the absence of HDAC11, host defenders such as neutrophils

viii and T cells are functionally more aggressive against intruders such as pathogen and cancer. However, the immune suppressors such as MDSCs became more suppressive.

The contradictory role HDAC11 played in the immune system may provide some insights for the assessment of the pharmacological value of HDAC11 and contribute to the development of novel immunotherapeutic strategies.

ix

Chapter 1: Background

1.1 Cancer

Cancer is the second leading cause of death in the United States and a major

health problem worldwide. There are 1,688,780 new estimated cancer cases and 600,

920 estimated cancer death in the United States in 2017. Prostate, lung & bronchus and

colon & rectum are the top three types of cancer incidence in male with estimated

incidence rate of 19%, 14% and 9% respectively. In women, breast cancer consist 30%

of projected cancer incidence along with other two leading cause lung and colon cancer.

In both sex, lung cancer appears to be the top projected cause of death in 2017 [1].

Overall, both cancer incidence and death rate are slowly decreasing since 2004 and 5-

year survival rate has increased by 20%, possibly benefit from the development of

treatments, introduction of cancer screening and more access to medical care among

all population [1]. However, the efficacy of current therapeutic strategies is limited by the

unresponsiveness and resistance from patients after treatment. The complexity of tumorigenesis remains a huge challenge to the development of therapeutics.

In their paper published in 2000, Hanahan and Weinberg proposed a model for six features that were shared by most even all human cancers: sustained proliferative signaling, evasion of growth suppression, ability to metastasize, replicative immortality,

1 ability to induce angiogenesis and resistance to cell death [2]. With new knowledge

gained by current developments in the cancer research field, this model was further re-

defined by Yousef Ahmed Fouad and Carmen Asnei with seven hallmarks by combining

a few common features together and adding three new hallmarks: metabolic rewriting,

an abetting microenvironment and immune modulation [3]. Interestingly, many recent

studies provide evidences indicting that many mechanisms for tumorigenesis are

shared among different hallmarks. Finding over-lapping areas cross cancer types can

benefit the development of cancer therapies in a long run.

1.1.1 Cancer and host immune system

The host immune response, which has been studied extensively for the past few

decades, can be broadly categorized as innate immunity and adaptive immunity. It is

well accepted that the adaptive immunity relies on the innate immunity during the

initiation and development of adaptive effector mechanisms. Upon pathogen recognition, cellular mediator for the innate immunity such as macrophages, neutrophils and nature killer cells migrate toward the location of infection and produce high level of cytokines and chemokines to direct the killing or phagocytosis of infected cells. The antigens

recognized by antigen-presenting cells such as macrophages and dendritic cells will

then be presented to the key components for adaptive immunity and direct the clonal

expansion of antigen-specific effector cells selected by receptor rearrangement [4,

5].

2 In both cancer patients and tumor bearing animal models, it is widely observed

the host immune system often silenced when encounter tumors. The theory of cancer

immune surveillance was accepted with the observation of the defensive machinery in

the host body that was associated with immune mediated rejection of tumors in mouse

models. However, despite the immune surveillance tumor maintained its capability of

continuous development. The phenomenon of cancer immunoediting, which includes

three stages as elimination, equilibrium and escape, can best summarize the interaction

between tumor and host immune system. The elimination phase can best describe the

process of host immune response against cancer: by recognition of tumor cells, the

innate immune cell undergoing maturation and migration followed by the presentation of

tumor antigen and priming of cytotoxic T lymphocytes, which triggers the homing of

these T cells to tumor site and directs the killing of tumor cells. The continuous

elimination of tumor cells can then trigger the tumor cells evolution by the selection of

cells with reduced immunogenicity and increased resistance. This process defines the

second phase: equilibrium. The final step of tumor immunoediting is involved with the

evasion of immune surveillance and elimination and eventually results in tumor

"escape" (reviewed in [6]).

During the phase of selection, tumor cells are undergoing multiple genetic and

epigenetic alterations toward a "suitable" phenotype that could potentially survive the

immune attack. Mechanisms generated by tumor cells include 1) reduced antigen recognition by alter either tumor cell surface marker expression or the effector T cells, 2) induce immunological ignorance and tolerance through tumor secreted immunosuppressive factors or recruitment of immunosuppressive cells such as myeloid-

3 derived suppressor cells and tumor associated macrophages, 3) reduce

cytokine/chemokine mediated cell death [3].

In order to evade the immune surveillance and promote its progression, tumor

cells generate multiple mechanisms to "build" or "re-edit" the local microenvironment

they were growing in. The tumor microenvironment provides physical support, nutrition

and chemical signals for the tumor growth. However, large amount of immune cells and

non-immune cells are recruited to the TMEs as well. Large amount of literatures had

reported the infiltration of T cells, myeloid cells and cancer-associated fibroblasts in the microenvironment. The crosstalk between these cells and tumor cells can significantly impact the effectiveness of cancer therapy. The infiltration of antigen-specific effector T cells in the TMEs could potentially provide immune defense against cancer and present as a good prognostic marker. However, the anti-tumor function of those T cells is often suppressed by either tumor cells or other immunosuppressive cells recruited by the tumor. The reprogramming of immunosuppressive factors in the TMEs will be discussed later in this chapter.

1.1.2 Hematopoiesis and abnormal myelopoiesis

Hematopoiesis plays a crucial role in the immune system in humans. The hematopoietic stem cells (HSCs) are responsible for the production of all lineages of blood cells at a daily basis. Upon stimulation with different kind of signals such as growth factor and cytokines, HSCs undergoes multiple steps of differentiation and commitment and eventually give rise to multiple lineages of cells. In the bone marrow,

4 common myeloid progenitor cells (CMPs) will be further differentiated into

Megakaryoblast, Proerythroblast, master cells and Myeloblasts, whereas common

lymphoied progenitor cells (CLPs) will be further differentiated into B, T lymphocytes

and lymphoid dendritic cells. The Myeloblasts will eventually give rise to

monocytes/macrophages, neutrophils, dendritic cells, basophils and eosinophils. At

steady state, hematopoiesis is strictly regulated by different genetic and epigenetic

modulations to maintain homeostasis of the host immune system. However, under

pathological conditions such as infection, HSCs will undergoing expansion and induce

the production of different kinds of blood cells to replace the cells that are "consumed"

during innate and adaptive immune responses.

During tumorigenesis, abnormal myelopoiesis could occur in response to a

persistent signal of growth factors (such as GM-CSF, G-CSF and M-CSF) produced by tumor cells or tumor induced inflammation [7]. As a result, large amount of immature myeloid cells will enter the blood stream and migrate to the sites of demand. As previously discussed, tumor cells can develop multiple mechanisms to evade immune surveillance such as induced immunosuppressive pathways. During this tumor induced emergency myelopoiesis, once being released to the blood, the immature myeloid cells will differentiated into functional suppressive MDSCs and enter the tumor microenvironment [8-10]. Moreover, besides growth factors listed above, cytokines such as IL-1β can also induce increased hematopoiesis in the bone marrow in tumor bearing mice, possibly through the release of increased level of serum IMCs induced by IL-1β

[11]. Interestingly, recent studies had demonstrated that tumor induced abnormal

myelopoiesis can be mediated by HDAC inhibition. Besides the direct cytotoxic effect of

5 HDAC inhibitors on cancer cells, HDACi has been shown to induce the cytotoxic activity of CD8+ T cells against cancer. However, administration of HDACi in vitro result in an enhanced bone marrow proliferation and functional MDSCs expansion [12]. To date, the mechanism of HDAC inhibition affect myelopoiesis is not fully understood yet. In chapter two and three of this dissertation, questions regarding to mechanistic role of HDAC11, the newest member of the HDAC family, in hematopoiesis under pathological conditions such as infection and cancer will be further addressed.

1.1.3 Current approaches for cancer immunotherapy

Currently many different types of immunotherapy are used to treat cancer. (1)

The monoclonal antibodies. Some mABs are designed to bind to specific surface receptors and function as an agonist or antagonist, which can potentially cause an immune response that eliminates cancer cells. Other types of mABs may bind to the surface of cancer cells and direct immune recognition or the delivery of a cytotoxic agent [13]. (2) Cytokine treatment. The infusion of cytokines such as interferons and interleukins could potentially induce the proliferation and responses of the immune cells.

However, it also proved to be associated with various side effects and limited clinical outcomes. (3) Adoptive cell transfer. T cells isolated from the tumor have the potential to recognize tumor cells and are actively against them. Ex vivo expansion of those tumor- specific T cells and re-introduce to the patients can restore the anti-tumor immune response in many cases. The T cells from patients can also be genetically modified before re-infuse into the patients. More recently, CAR-T cell therapy has been approved

6 by the Food and Drug Administration (FDA) in treatment of ALL. (4) Vaccine treatment.

Cancer vaccines such as polypeptide based, whole cell, or viral vector can work against cancer by induce the responses by host immune system.

One challenge for modern cancer immunotherapy such as adoptive T cell transfer is to overcome the immunosuppressive microenvironment modulated by tumors.

The suppressive feather of the TMEs is associated with multiple factors such as cancer types and disease stages, as the host immune system is possibly compromised with advanced disease. Targeting the suppressive cells/factors in the TMEs or giving the treatment at an earlier stage might provide a better outcome in combination with these therapies.

1.2 Tumor microenvironment

Cancer cells are the crucial components to form a malignant tumor. But the malignant phenotype of cancer cells cannot be achieved without the surrounding cells in the local environment where primary or metastasis tumor mass was formed. Together with tumor cells, the non-malignant cells such as lymphocytes, myeloid cells, fibroblasts, along with the extracellular matrix, blood vessels and other supporting structures composed tumor microenvironment [14]. The infiltrated non-malignant cells in the tumor microenvironment displayed a dynamic and tumor promoting function during the initiation, progression and metastasis of many solid tumors [15]. Both tumor cells themselves and infiltrated immune cells can release a wide range of different cytokines, chemokines and growth factors which can contribute to the tumorigenesis, recruitment

7 of immune cells, as well as suppression of T cell function. The cross talk in between

tumor cells and infiltrated cellular and non-cellular components in the microenvironment

formed a dynamic network and brought huge challenge for therapeutic targeting the

tumor microenvironment.

1.2.1 Tumor infiltrated T lymphocytes

Studies had documented evidence of many different T lymphocyte populations

infiltration in tumors and the draining lymphoid organs. The prognostic significance of

those tumor infiltrated T cells is in debate considering the functional diversity of this

heterogeneous population of T cells when interacting with tumor cells or other cellular

components in the tumor microenvironment.

Higher number of infiltration of cytotoxic CD8+ memory T cell which have the

capability to recognize antigen and kill tumor cells are generally correlate with good

prognostic [16]. CD4+ T helper 1 cells (Th1, produce IL-2 and IFN-γ), which support

CD8+ T cell function, are also considered as good prognostic when largely infiltrated in

the tumors [16]. On the contrary, CD4+ T helper 2 cells (Th2, produce cytokine such as

IL-4, IL-13 and IL-5)) and immnosuppressive regulatory T cells (Tregs express surface marker Foxp3 and CD25) in the tumor microenvironment are believed to be tumor promoting [16, 17]. Additionally, higher number of Tregs in the blood of diffuse large B- cell lymphoma is associated with poor prognostic, but Tregs can be tumor suppressive in Hodgkin's lymphoma patients [18-20].

8 However, multiple mechanisms had been identified to discharge Cytotoxic T

cell's anti-tumor function in the microenvironment. The key effectors that contribute to the immune dysfunction include tumor cells, suppressive immune cells and stromal cells. Tumor cells often over-express or down-regulate certain cytokines to manipulate

immune responses. For instance, over-expression of CCL2 in colorectal cancer result in increased recruitment of tumor associated macrophages that contribute to cancer progression [21]. Enhanced secretion of chemokine ligand CCL22 by breast cancer is associated with the infiltration of regulatory T cells [22, 23]. Lacking expression of

CCL2, CCL3 CXCL5, CXCL10 in metastatic melanoma can forbid antigen-specific T cell infiltration [23, 24]. Besides chemokines, tumor cells can up-regulate checkpoint proteins such as cytotoxic T lymphocyte antigen-4 (CTLA-4) and programed cell death-

1 (PD-1) on tumor infiltrated T cells, which are originally part of the normal suppressive mechanism during immune response, result in down-regulation of those T cell response and exhaustion [25].

1.2.2 Myeloid-derived suppressor cells

Myeloid-derived suppressor cells are defined as a heterogeneous population of inhibitory immune cells that are expanded result from abnormal myelopoiesis and neutrophilia in various cancers in murine models and humans [26]. MDSCs were first observed in the 1980s and were called "nature suppressor cells" characterized with its inhibitory ability to T cell proliferation and cytotoxic T cell generation. They were soon discovered in multiple cancer types such as head and neck, as well as murine animal

9 model bearing spontaneous and transplanted tumors [27, 28]. Researchers had

struggled how to term this special population of cells and until 2007 the terminology of

"myeloid-derived suppressor cells (MDSCs)" was finally accepted [29].

1.2.2.1 The origin of MDSCs

Based on currently knowledge, MDSCs are believed to originate from the bone marrow in both human and mouse (a small amount of MDSCs are believed to originate from spleen in mouse). They are derived from the hematopoietic stem cells to the common myeloid progenitor and the granulocyte/macrophage progenitor cells. During steady state hematopoiesis, these GMP cells usually further commit to granulocytes and macrophages. However, under pathological conditions such as cancer progression

(sometimes infection), the GMPs will further differentiated into MDSCs in bone marrow, blood, lymph nodes, spleen, tumor site and metastasis, with an expansion of more than

20% of total cells [30]. Originally, MDSCs are defined as cell express surface marker

CD11b and Gr-1 (Ly6G/Ly6C). However, during normal hematopoiesis, CD11b+Gr-1+

cells constitute 20 to 30% of total cells in the bone marrow and 2 to 3% in the spleen.

The surface markers are not exclusively expressed on MDSCs, which make it

necessary to include the functional characterization as part of the strategy to identify

this special population.

10 1.2.2.2 MDSCs expansion

Several growth factors were demonstrated to affect the MDSC expansion:

granulocyte-macrophage colony-stimulating factor (GM-CSF), granulocyte colony- stimulating factor (G-CSF) and macrophage colony-stimulating factor (M-CSF). During myelopoiesis at steady state, GM-CSF, G-CSF and M-CSF are believed to play a crucial role in the maturation, proliferation, recruitment and survival of granulocytes and monocytes. When treated with GM-CSF and/or G-CSF in vitro, bone marrow precursor cells acquired a similar surface phenotype and function to MDSCs induced by cancer

[31, 32]. Thus, it is not surprising that the high secretion of GM-CSF and G-CSF from cancer cells is associated with MDSCs expansion and infiltration. Long-term exposure of high level of GM-CSF and G-CSF produced by cancer cells affect the generation, maintenance and survival of MDSCs in cancer patients [33]. However, there's no evidence showing that short administration of GM-CSF to patients has the same effect

[34].

1.2.2.3 Subsets of MDSCs

Recently, two major subsets of MDSCs have been identified based on their morphology and surface markers expression: monocytic MDSC (M-MDSC) and granulocytic MDSC (G-MDSC, also called polymorphonuclear PMN-MDSC) [35, 36]. In mice, M-MDSCs are characterized as CD11b+Ly6G-Ly6Chigh while G-MDSCs as

CD11b+Ly6G+Ly6Clow [36-38]. However, in cancer patients, the definition of phenotype

of human MDSCs is unclear. By far human M-MDSCs are defined as

11 CD11b+CD14+CD33+HLA-DR-/lowCo-receptor-/low and G-MDSCs as

CD11b+CD15+CD33+Lin-HLA-DR-/low expressing cells [8, 39].

Functionally, M-MDSCs are believed to be highly immunosuppressive in both

antigen specific and non-specific manner, whereas G-MDSCs' immunosuppressive

effect is moderate and its promotion to T cell tolerance is antigen-specific. Monocytic

MDSCs express high level of both immunosuppressive enzymes arginase and inducible

oxide synthetase (iNOS), but could not produce high level of reactive oxygen species

(ROS). On the contrary, granulocytic MDSCs are capable of the expression of high level

of arginase and the production of high level of ROS, but fail to express iNOS [26, 40].

Besides increased expression of Gr-1 surface marker on both populations, M-MDSCs express high level of IL-4 receptor alpha (IL-4α) compare with G-MDSCs [41]. However, the low intensity of IL-4α on and its inconsistent expression pattern on in different cancer hosts forbid it to become a good surface marker to distinguish the subsets of

MDSCs [42, 43].

1.2.2.4 Mechanisms regulating MDSCs biology

Two major questions regarding MDSCs biology are: 1), what is the mechanism(s) that regulates MDSC generation and expansion; 2), what controls their tumor promoting function. Recent studies had established multiple signal pathways that were regulating

MDSC expansion and function. The transcription factors involved with those pathways often overlap or have impact on each other and formed a complicated signal network that benefit MDSCs suppressive capability.

12 The signal transducer and activator of transcription (STAT) family had been

demonstrated to play an important role in different mechanisms. Activation of STAT3

can directly promote proliferation; prevent cell apoptosis and differentiation in MDSCs,

through over-expression of Bcl-xL, c-myc and cyclin D1 [30]. Another pathway STAT3

involved with is the calcium-binding pro-inflammatory protein S100A8 and S100A9 [44].

Upregulation of S100A8 and S100A9 by STAT3 result in MDSCs accumulation in mice

[9, 44]. Activation of STAT3 was also proven to directly relate with up-regulation of ROS

production in MDSCs, possibly through up-regulation of the NADPH oxidase (Nox2)

components P47phox and gp91phox [9]. Notable, there's new evidence showing that

STAT3 activation result in an up-regulation of transcription factor CCAAT-enhancer- binding protein beta (C/EBPβ), in responding to G-CSF in vitro [31]. C/EBPβ plays a

crucial role in the differentiation and immunosuppressive function of BM-derived and

tumor-induced MDSCs. Mice lacking C/EBP-β in the bone marrow compartment

appeared to lose the ability to differentiate from IMCs into pathologically active MDSCs, possibly through a reduction of arginase 1 and Nos2 proteins expression [31]. In addition to STAT3, other members of the STAT family had shown to be associated with

MDSCs function. In response to IL-1β or IFN-γ, STAT1 can up-regulate iNOS and arginase activity, leading to a functionally more suppressive MDSCs phenotype [45, 46].

Some studies reviewed that STAT5 and STAT6 played a crucial role to MDSC survival

and function through multiple pathways.

One mechanism of the suppressive function in MDSCs is mediated by Arginine

metabolism. Arginine is a non-essential amino acid for adult human and its metabolism

is largely associated with immune response. L-Arginine deprivation has no effect on

13 primary T cells at steady state, from both mouse and human. Once activated in L-

Arginine free culture medium, T cells appeared to have decreased expression of CD3ζ

chain and down-regulation of NFκB-p65 and Jak-3, as well as a reduced proliferation

and inability of IFN-γ production [47, 48]. Arginine can be metabolized by a few

enzymes such as arginase, nitric oxide synthase (NOS), arginine:glycine

amidinotransferase (AGAT) and arginine decarboxylase. In MDSCs, higher expression

of Arg1 and Nos2 are often detected among different subsets that are related with

MDSCs to T cell suppression. There are two isoforms of arginase, Arg1 and Arg2.

Arginase 1 mainly expresses in myeloid cells and hepatocytes. Arginase 2 can be found

in tissues such as kidney, brain and small intestine [48]. Arg1 can convert L-arginine to

urea and L-ornithine, whereas Nos2 can generate nitrite oxide (NO) and L-citrulline

when utilize L-arginine. Increased NO production by MDSCs infiltrated into tumors is associated with T cell suppression through interfering T cell and APC communication, inhibit T cell signaling downstream of IL-2R and induce T cell apoptosis [49-51]. Up-

regulation of Arg1 and Nos2 in MDSCs are mediated through different signal pathways.

Activation of STAT6 and up-regulation of transcription factor C/EBPβ and STAT3 are

associated with increased Arg1 expression in MDSCs. Additionally, multiple cytokines

and growth factors such as IL-4, IL-10, IL-13, TGF-β and GM-CSF can induce the

expression of Arg1 as well [48]. Moreover, Nos2 production can be induced in response

to IFN-γ, TNF-α and IL-1β as mentioned before.

In addition to up-regulation of Arg1 and Nos2, increased expression of NOX2, the

catalytic subunit of NADPH oxidase can result in increased level of ROS in granulocytic

14 MDSCs, mediating T cell inhibition through downregulating CD3ζ chain expression, reducing cytokine production and impairment of the differentiation of MDSCs [52].

Besides directly suppress T cell function, MDSCs was found to induce the development of CD4+CD25+Foxp3+ Tregs in a colon carcinoma mouse model [53].

Additionally, a few inhibitory methods that are used to reduce MDSCs function were found to contribute to reduce Treg function and expansion [54, 55].

1.2.2.5 Clinical evaluations of MDSCs

The important role of MDSCs has been acknowledged for tumor initiation, progression and immune evasion. However, in clinic, the expansion of MDSCs has been correlated with advanced stages in multiple cancer types such as head and neck squamous cell carcinoma, none small cell lung cancer (NSCLC), hepatocellular carcinoma (HCC), bladder cancer, pancreatic cancer and hematological malignancies such as non-Hodgkin lymphoma (reviewed in [56]). Moreover, besides tumor progression stages, MDSCs level also correlates with patient response to therapy and surgery. Clinical data reviewed that circulating MDSCs level in the blood could serve as a predictive and prognosis marker. An example is in stage IV breast or colorectal cancer patients, high circulating MDSCs number is associated with poor prognosis (reviewed in

[56]). Higher number of blood circulating MDSCs at baseline prior to chemotherapy also correlated with a shorter overall survival (reviewed in [56]).

15 1.2.2.6 Targeting MDSCs in cancer

The functional characterization and clinical correlation study reviewed MDSCs as

an important factor in cancer in general and reflect them as a potential target for

therapeutic approaches. The fact that MDSC is primarily correlate with T cell

suppression indicate the potential to directly target this population or as adjuvant with

current immunotherapy. As reviewed by many researchers, blocking MDSCs

generation, recruitment or suppressive function, as well as converting MDSCs to APCs

could be beneficial as potential therapeutics. One in vivo study using nitroaspirin (NCX-

4016) had observed restoration of T cell responsiveness and enhanced efficacy of

cancer vaccination, possibly through inhibition of Arg1 and iNos activity [57].

Administration of all-trans retinoic acid (ATRA) or vitamin D3 result in differentiation of

MDSCs toward matured myeloid cells thus improve the efficacy of anti-tumor vaccination [58]. Some earlier generations of chemotherapeutic agents such as 5-

Fluorouracyl showed to affect monocytic MDSCs apoptosis in animal models [59, 60].

Inhibition of CSF1/CSF1R by using CSF1R antagonist and anti-CSF1R mAB (RG7155) affects monocytic MDSCs differentiation and recruitment in some clinical trials [61].

Although increasing therapeutics have been designed to target MDSCs through

different pathways, more investigation should be dedicated to assess the safety,

effectiveness and side effects of the new agents before entering clinical trial. Moreover,

the complexity of the signal pathways regulating MDSCs function brings huge challenge

to targeted therapeutics. A better understanding of the network mediating MDSCs

biology will likely to benefit cancer treatment in a long run.

16

1.2.3 Tumor associate macrophages

Tumor associate macrophages (TAMs) are a major leukocyte population

infiltrated in tumors. Depend on the microenvironment; macrophages could acquire

different phenotypes associated with different functions. Classically activated

macrophages (M1 macrophages) and alternatively activated macrophages (M2

macrophages) are often considered to be the two major polarized phenotypes of

macrophages. M1/M2 macrophages are driven from different cytokines. IFN-γ or LPS

can induce M1 macrophages that are generally considered to be pro-inflammatory. M2

macrophages can be polarized in response to IL-4 and IL-13 and are characterized with

anti-inflammatory phenotype. Tumor associated macrophages are usually linked with

M2 macrophages. However, the transcription profile of TAMs is quite distinct from both

phenotypes [62, 63].

Unlike regular macrophages, which have the potential to eliminate tumor cells, tumor associate macrophages are one of the major contributors to many stages of tumor progression. The pro-tumorigenic effect of TAMs is largely reflected on their

involvement with tumor cell migration, invasion and metastases. The

immunosuppressive effects of TAMs and MDSCs are overlapping in many manners. For

instance, both TAMs and MDSCs can suppress T cell function in an antigen-specific

and non-specific manner. TAMs and MDSCs can both induce CD4+CD25+Tregs

generation and recruitment [64, 65]. Similar to MDSCs, TAMs shared high enzymatic

activity for L-arginine consumption that deploys one of the most powerful suppressive

17 features in regulating T cell response and proliferation [66]. In addition to immune suppression, TAMs and MDSCs both played a crucial role in promoting tumor angiogenesis by secreting VEGF, CXCL8/IL-8 in response to hypoxia [56].

1.2.4 Other cellular and non-cellular components in the tumor

microenvironment

Besides T lymphocytes and suppressive myeloid cells, other types of immune cells can be found at the invasive margin of some tumors and/or draining lymph nodes as well. The infiltration of natural killer cells (NK cells) and natural killer T cells (NKT cells) can be found in some cancer types and are associated with a good prognosis

[67]. Dendritic cells (DCs) and tumor associated neutrophils (TANs) are also reported to infiltrate tumors. However, the primary function of DCs and TANs that are related with antigen processing and presenting are usually defective. Instead, when interact with malignant cells or local microenvironment, they appear to promote tumor progression by suppressing T cell response or enhancing tumor growth and angiogenesis. In addition, the infiltration of B cells in medullary ductal breast cancer and high-grade serous ovarian cancer was associated with good prognosis [68, 69]. However, their function can be switched to tumor promotion by inhibit tumor specific cytotoxic T cell response

[70].

Other cellular or non-cellular components in the tumor microenvironment had been shown to play crucial roles in cancer progression. Myofibroblasts in TME, also known as cancer-associated fibroblasts (CAFs), are associated with promotion of

18 cancer cells growth and survival, maintaining the immune suppressive

microenvironment as well as inducing epithelial-mesenchymal transition (EMT) through secretion of growth factors and chemokines [71]. Adipose cells and tissue are associated with secretion of large amount of cytokine, chemokine and hormone-like

factors to support and maintain a pro-inflammatory microenvironment, contributing to

tumor progression and immunosuppression. In addition, the non-cellular components in

TMEs, extracellular matrix (ECM), contain cytokines and growth factors secreted by

tumor cells and stromal, which contribute to tumor growth and recruitment of

immunosuppressive cells [71]. ECM also provides a physical scaffold for all cells in the

tumor microenvironment, helping maintain tumor homeostasis.

1.2.5 Strategies of targeting the tumor microenvironment

The cellular and non-cellular components of the tumor microenvironment permit

and promote tumor progression in many different aspects and a lot of them were found

over-lapping during the cross talk between each other. Therapeutic strategies can be

developed to target both cellular and non-cellular components in the TMEs.

(1) Targeting immune cells. Chronic inflammation induced by tumor infiltrated

macrophages, neutrophils and master cells is usually associated with cancer

progression. Depend on the polarization phenotype, immune cells can exert

either anti- or pro-tumor effect. The presence of M1 macrophages, N1

neutrophils, Th1 CD4+ helper T cells and cytotoxic CD8+ T cells sometimes

indicate a good prognostic or favorable to immune therapy. However, these

19 immune cells possess potential anti-tumor effect that is often found to be suppressed through different pathways. On the other hand, cells with immunosuppressive phenotype such as tumor associated macrophages,

MDSCs and regulatory T cells either promote tumor progression directly or suppress immune response. Thus, targeted therapeutics could be considered based on the unique function of a particular cell type. For instance, blocking the recruitment or reprograming suppressive cells in the TMEs may potentially reduce the pro-tumor effect and induce immune response against tumor cells.

The cytotoxic CD8+ T cells present in the tumor is often not sufficient enough against tumor cells. Additional adoptive transfer of antigen-specific CD8+ T cells was proved to have positive effects on some patients. However, the over-all suppressive microenvironment those T cells encountered may potentially affect the anti-tumor response. Adding a suppressor cell blockade may potential benefit the effect for the adoptive transfer therapy. Anti-inflammatory drug have been found to reduce tumor incidence and could potential serve as an anti- tumor agent, however, its side effects and specificity remains questionable.

(2) Targeting none-immune cells in the TMEs. Cancer associated fibroblasts

(CAFs) are involved with tumor progression and metastasis by providing support to tumor cell growth and cell-cell interaction in TMEs, induce angiogenesis and the presence of CAFs usually associated with poor prognosis in many cancers. Extensive cross talk between tumor cells, CAFs and immune cells in the TMEs was observed among different cancers, indicating that CAFs possessed a therapeutic value. Although a few clinical studies had failed to

20 either achieve clinical benefits or end up reducing survival rate, further understanding will contribute to the development of targeted therapy against

CAFs.

(3) Targeting non-cellular component in the TMEs. The extracellular matrix in

TMEs facilitates support for the interaction between cancer cells and host cells.

In addition to the physical network, ECM creates an environment contains different chemical signal with the ability to regulate the oxygen level, pH value and interstitial pressure for the TMEs [72] that affects tumor progression, which may provide multiple potential targets for cancer therapy.

(4) Targeting angiogenesis. Extensive interests and developments on tumor angiogenesis during the past 40 years contribute to the development of therapeutics targeting this specific hallmark of cancer. Many clinical trials have been conducted using different kind of agents such as anti-VEGF antibody and antiangiogenetic inhibitors. However, the feedback of antiangiogenetic drugs turned to be modest with a lower responding rate [73], possibly through tumor dependent or independent resistance. Alternative strategies proposed by cancer researchers are using antiangiogenic drugs as adjuvant therapies in combination with other therapies such as chemotherapy, conduct trials for patients at early stage of tumor development and developing therapeutics against VEGF-independent angiogenic factors (reviewed in [72])

21 1.3 Histone deacetylases

In eukaryotes, DNA is tightly compacted with an octamer of four (2

H2A/H2B dimers and a H3/H4 tetramer) to form the nucleosome, subunit of chromatin.

This highly dynamic protein-DNA complex prevents the accessibility of transcription factors in resting cells whereas made available through chromatin remodeling during post-transcriptional regulation. Epigenetic modification, including DNA methylation, phosphorylation, ubiquination and acetylation, could alter the chromatin architecture thus influence the transcriptional regulation. Although DNA methylation is well studied, acetylation of histones had gained increasing amount of interest for scientific investigators in recent years.

Two groups of enzymes have been recognized to modify histone acetylation status based on their function. Histone acetyltransferase (HATs), function as epigenetic writers, transfer acetyl group to lysine residues on histones (and other acetyl-lysine containing proteins) and allow accessibility of transcription machinery to a “relaxed” chromatin structure and up-regulate a particular . On the contrary, the epigenetic eraser histone deacetylases (HDACs) remove acetyl group from histone tails, form a condensed chromatin and make it unfavorable to DNA binding proteins and repress transcription [74, 75].

1.3.1 Classification of HDACs

Presently in humans, there are eighteen known HDACs so far with differences in amino acid sequence, enzymatic domain structure and tissue specific expression

22 pattern. Besides the nicotinamide-adenine-dinucleotide (NAD+)-dependent , eleven out of these eighteen zin-dependent HDACs were found with conserved deacetylase domain and divided into four classes: class I HDACs (including HDAC1,

HDAC2, HDAC3 and HDAC8); class II (HDACs 4, 5, 6, 7 and 9) and class IV with a sole member HDAC11. Class II HDACs were further divided into two sub-classes: class IIa includes HDAC 4, 5, 7 and 9; class IIb includes HDAC6 and HDAC10 which both have two catylytic sites. Class I, IIa, IIb and IV HDACs are grouped as classical HDACs [76,

77].

The predominant localization of almost all HDACs was found to be the nuclear through a nuclear localization signal (NSL) or the co-localization with other proteins.

Class I HDACs are mostly found exclusively in the nucleus except for HDAC3, which contains both a nuclear import and export signal. HDAC11 is primarily located in the nucleus, however, using co-immunoprecipitation, both endogenous and over-expressed

HDAC11 can be detected in the cytoplasm when associated with HDAC6 [78, 79]. Class

II HDACs can shuttle in and out of the nucleus, either induced by certain signal or depend on the splice variants [76].

1.3.2 HDAC11

Histone deacetylase 11 was first cloned and identified in 2002 by Gao and colleagues. The molecular weight of HDAC11 is 39 kDa with a total sequence of 347 amino acid consisting nine exons and eight introns [79]. In humans, HDAC11 protein is encoded on chromatin 3p25.2 whereas on chromatin 6 in mouse [79]. As the smallest

23 member in the HDAC family, HDAC11 contains all blocks of conservative sequence in its catalytic domain that are identified to be important for the enzymatic activity for deacetylase function among other HDACs [79]. Phylogenetic analysis had reviewed

HDAC11 is more closely related to class I HDACs and the prokaryotic AcuC protein than class II HDACs [79]. However, HDAC11 shared an extremely low similarity of its structure to class I, II and III HDACs and was granted with its own class, class IV [79].

The expression of HDAC11 characterized in various tissues and cancer cell lines. Northern blotting and mRNA analysis from Gao, et al reviewed that HDAC11 expression was highly tissue specific, with a higher expressing signature in brain, heart, skeletal muscle, kidney and testis, suggesting that its function may be tissue specific

[79]. Higher level of HDAC11 was also detected in several human malignant cell lines such as rhabdomyosarcoma muscle tumor cell line [79]. An in vitro deacetylase activity assay using FLAG-HDAC11 showed that HDAC11 possessed catalytic activity shared by other family members, but in a much lower manner [79]. However, using a synthetic peptide in vitro to measure the activity might not reflect the true deacetylase ability of endogenous HDAC11.

Using same FLAG-HDAC11 over-expression model, Gao's group identified a physical association between HDAC6 and HDAC11 293 cells [79]. Both calss I and II

HDACs had been previously proved to function in protein complexes including transcription repressors and other proteins from the HDAC family. However, besides

HDAC6, no evidence indicates that HDAC11 is physically involved with other HDAC family members. HDAC11 was previously found to localize in the nucleus. However, recent study detected the interaction of HDAC6 and HDAC11 in both cytoplasmic and

24 nuclear compartment in murine and human macrophage or promonocytic cell lines,

through the N-terminal domain of HDAC11 and C-terminal domain of HDAC6 [78].

1.3.2.1 HDAC11 in APCs

The functional role of HDAC11 in immune cells was largely undefined until

Villagra in our group first found out that this molecule played as a transcription repressor of IL-10 expression in macrophages. In response to LPS, macrophages with HDAC11 over-expression result in decreased IL-10 expression, possibly through alteration of the phosphorylation of histone H3 in the proximal region of IL-10 promoter [80]. Further investigation by the authors showed that HDAC6 and HDAC11 played opposing roles in regulating IL-10 expression in APCs. The authors had demonstrated that over- expression of HDAC6 results in an increased IL-10 production in macrophages [78].

Interestingly, the physical interacted HDACs were found to be recruited to the same region in the IL-10 promoter, suggesting the opposite role of HDAC6 and HDAC11 played in regulation IL-10 expression might related with their dynamic interaction under stimulation [78].

Recently, another group had demonstrated that HDAC11 up-regulation result in a down-regulation of IL-10 in Kupffer cells isolated from alcohol-fed mice, possibly through alcohol metabolites (ALDH) and NF-κB pathways [81]. Furthermore, decreased

IL-10 production was observed in dendritic cells when HDAC11 was knocked down using shRNA.

25 1.3.2.2 HDAC11 in T lymphocytes

The functional role of HDAC11 in T lymphocytes was characterized by two recent

publications. Woods and Woan from our group had previously observed an increased

effector function by T cells lacking HDAC11. Using a HDAC11 knockout mouse model,

the authors found that in the absence of HDAC11, CD8+ T cells displayed increased

proliferation rate and surface expression of effector molecules such as granzyme B and

perforin [82]. Both HDAC11KO CD4+ and CD8+ T cells produced higher level of proinflammatory cytokines in both antigen dependent and independent manner. In addition, HDAC11KO T cells were less susceptible to tolerance induction and experience less suppression from Tregs [82]. Furthermore, in vivo study demonstrated an enhanced anti-tumor effect of T cells lacking HDAC11 through adoptive transfer therapy [82]. One proposed mechanism by the authors was HDAC11 mediate the hyperactive phenotype of T cells through alteration of chromatin structure in the promoter region of transcription factors Eomes and Tbet. Using Chromatin-

immunoprecipitation, the presence of HDAC11 in both promoters of Eomes and Tbet

was determined, along with a decreased acetylation signature of histone 3 in both

promoters in resting WT T cells [82]. Meanwhile, another study demonstrated that

deletion or pharmacologic targeting of HDAC11 using a HDAC11 inhibitor (JB3-22) could enhance the suppressive function of Foxp3+ Tregs and promotes Treg-dependent

allograft survival [83].

26 1.3.2.3 HDAC11 in cancer

Upon its identification, aberrant up-regulation of HDAC11 has been reported in a few cancer cell lines including rhabdomyosarcoma (SJRH30), colorectal carcinoma

(HCT116), urinary bladder carcinoma (T24), non-small cell lung carcinoma (H1299) and oxteosarcoma (SJSA-1) [79]. Recently, another report pointed out that HDAC11 depletion by siRNA in HCT-116 colon, PC-3 prostate, MCF-7 breast and SK-OV-3 ovarian cell lines resulted in induced apoptosis and reduced metabolic activity of the cancer cells, however, such effects could not be reflected on normal cells [84]. Knocking down of HDAC11 in Hodgkin's lymphoma cell lines result in a significant enhanced surface expression of OX40L, which is essential for T cell activation and memory formation when engage with OX40 receptor B [85]. Additionally, a higher expression of

HDAC11 that observed in pituitary tumor tissue, which appears to be correlated with down-regulation of tumor suppressor P53, possible through the deacetylation of HEY1

[86]. Another study in neuroblastoma demonstrated that HDAC11 deletion triggered cell death through apoptotic programs [87].

1.3.2.4 Other functions of HDAC11

Earlier studies showed that HDAC11 control DNA replication. Through binding

Cdt1 during S-phase, HDAC11 deacetylates this molecule and inhibits its ability to induce chromatin unfolding thereby limits DNA replication [88]. A recent study had

demonstrated that HDAC11 can deacetylase Cdc25, a key regulator of cell cycle, via

direct protein-protein interaction [89].

27 In addition, expression of HDAC11 was observed at the highest level in mouse oligodendrocytes and its expression was increased during brain maturation after birth

[90]. One study by Liu et al had demonstrated that using HDAC inhibitor (TSA) or silencing HDAC11 expression by siRNA result in an increased histone 3 lysine 9 and lysine 14 (H3K9/K14) acetylation within the myelin basic protein (MBP) and proteolipid protein (PLP) gene promoters in the oligodendrocyte cells, leading to an abruption of their maturation and development [91].

Furthermore, HDAC11 has also been found to be involved in the regulation of myc gene along with vitamin D3 [92]. Interestingly, another recent study found out that

Vitamin D3 could induce the binding rate of Vitamin D receptor (VDR) and HDAC11 to the promoter loci of tight junction protein genes, result in the repressing of gene expression and the dysfunction of epithelial barrier [93].

Although attention and knowledge of HDAC11 is expanding during the past two decades, the full functional signature of HDAC11 still needs to be further defined. More understanding of the mechanistic role HDAC11 played in cancer and other diseases will contribute to further explore its potential as therapeutic targeting in those fields.

1.3.3 Other HDACs

HDAC1, 2, 3, 5, 6, 7 and 10 can be detected in all tissues whereas HDAC8 and 9 are mostly expressed in tumor tissues and cells. However, HDAC4 expression is non- detectable in somatic tissues but can be found in embryonic muscle tissue, which indicate that this HDAC is not essential [74].

28 HDAC 1 and 2 only display enzymatic activity within protein complexes such as

Sin3, nucleosome remodeling and deacetylating (NuRD) complex. Multiple cytokines,

transcription factors and growth factors are found to be downstream targets of HDAC1,

2 and 3. Class II HDACs possessed diverse functions among the family. Among all the

class II members, HDAC6 is the most studied and one of the two HDACs contained two deacetylase domains. HDAC6 was found to regulate various pathways related with autoimmune disease and cancer, which made it attractive pharmacological targets.

A typical characteristic of human cancer is the aberrant regulation of DNA methylation and histone modification, especially histone acetylation. It is not surprising that HDACs are believed to play an important role in cancer progression. Increased

HDAC activity is observed in many cancers possibly through recruitment of HDACs to specific promoters of tumor suppressor genes. Moreover, up-regulation of HDACs expression is observed in many cancers. For instance, HDAC1 overexpression is found in breast, colon, gastric, and prostate cancer [94-97], whereas HDAC2 is over- expressed in cervical, colorectal, and gastric cancer [98, 99]. HDAC3 up-regulation is reported in colon cancer and HDAC6 in breast cancer [95, 100]. These findings indicate that HDACs could be potential targets for cancer treatment.

1.3.4 HDAC inhibitors

HDAC inhibitors (HDACi) are most considered as promising cancer therapeutics along with its crucial role in treating immunological diseases such as viral infection and neurological disorder [101]. Besides the ability to inhibit the enzymatic activity of

29 HDACs, HDAC inhibitors also have the potential to target non-histone proteins in order

to inhibit angiogenesis, cell proliferation and also stimulate cell-cycle arrest [102, 103].

Based on the chemical structure, the HDAC inhibitors can be classified into hydroxamic

acid, benzamides, aliphatic acid and cyclic peptides, electrophilic ketones and

miscellaneous compounds [104].

In the past, the most commonly known HDAC inhibitors usually target multiple

HDACs. This can bring challenges to identify whether the effect of those inhibitors is

due to inhibition of a particular HDAC activity or in combination of multiple HDACs.

Moreover, several HDACs are believed to interact or depend on each other to achieve a

biological outcome, which suggest that even a putative specific HDAC inhibitor could

potentially develop broader effect than anticipated. Currently, HDACi is observed to

regulate various cellular or molecular effects in antitumor, immunological and

neurological responses.

A lot of questions remained unanswered in regarding to the direct anti-tumor effect of HDAC inhibitors. However, it is generally acknowledged that HDACis directly

effect on tumor cell through multiple pathways such as: 1) induced cell death through

induction of apoptosis, enhanced production of ROS, accumulation of DNA damage, 2)

induced cell cycle arrest, 3) induced senescence, 4) reversal of differentiation blocked

by fusion protein in AMLs, 5) induction of autophagy and 6) enhanced tumour

immunogenicity such as antigen-presenting capacity (reviewed in [75]). A few HDAC

inhibitors have been approved for cancer therapy by the US Food and Drug

Administration (FDA) in recent few years, namely suberoylanilide hydroxamic acid

30 (SAHA, 2006), romidepsin (2009) and belinostat (Beleoda1, 2014), in treatment of T-cell

lymphoma[105, 106] [107].

Many studies demonstrated that HDACs expression and function influence the

immune response under multiple pathological conditions including cancer. Considering

the wide spectrum impact of HDACis on different aspects when used as an oncology

drug, it could potentially achieve its best effect if a particular HDACi could target tumor

cells and induce anti-tumor immune response simultaneously. A few recent studies

have shown enhanced tumor antigenicity after treatment with HDAC inhibitor. For

instance, (AML), human neuroblastoma and mouse

plasmacytoma cells treated with HDACis such as TSA, trapoxin A or sodium butyrate

result in an up-regulation of MHC class I and MHC class II proteins, CD40, CD80, CD86

as well as adhesion molecule-1 (ICAM1) [108, 109]. Moreover, HDACi such as TSA,

SAHA and sodium butyrate has been shown to induce the expression of MHC class I

related chain A (MICA) and chain B (MICB), which are ligands for natural killer group 2

member D activating receptor on NK, CD8+ T cells, result in increased tumor-targeted

destruction [110, 111]. Additionally, TSA can induce the proliferation of cytotoxic CD8+

T cell and NK cells through enhanced expression of MHC class II and CD40 on B16

melanoma cells [112]. The antitumor immunity by HDACi SAHA could also result in decreased serum level of pro-inflammatory cytokines such as TNF-α, IL-1 and IFN-γ in an allogeneic GVHD model [113].

New HDACi compounds have been developed rapidly during the past few years.

However, the mechanisms for HDAC inhibitors to augment tumor rejection through either direct target or immunomodulatory effect are not fully understood by far. As for

31 the newest identified member of the HDAC family, only a handful of inhibitors have been reported to target HDAC11 with an unknown specificity feature. Recent work from our group and other laboratory had uncovered a novel regulatory role of T cell function by

HDAC11, implicated its potential therapeutic value. However, an earlier study from our lab demonstrated an opposite role of HDAC11 by enhancing the suppressive ability of

MDSCs, result in a pro-tumor effect. Given the complexity of the immunomodulatory role played by HDAC11, the next two chapters of this dissertation will be dedicated to present our recent work on how HDAC11 regulating immune response under different pathological conditions.

32

Chapter 2: Essential Role for Histone Deacetylase 11 (HDAC11) in Neutrophil

Biology1

2.1 List of Abbreviation

APCs: Antigen Presenting Cells

APL: Acute Promyelocytic Leukemia

ATCC: American Type Culture Collection

ATRA: All Trans-Retinoic Acid

CBA: Cytometric Bead Arra

CBC: Complete Blood Count

ChIP: Chromatin Immunoprecipitation

CXCL2: Chemokine (C-X-C motif) Ligand 2, Also known as Macrophage Inflammatory

Protein 2-alpha (MIP2-a)

CXCR2: Chemokine (C-X-C motif) Receptor 2, also known as Interleukin 8 Receptor

Beta (IL8Rb)

1 This chapter has been previously published (Chen J, Sahakian E et al. J Leukoc Biol. 2017 Aug; 102(2):475-486.) and utilized with permission from the publisher.

33 DCs: Dendritic Cells

E. coli: Escherichia Coli

eGFP: Enhanced Green Florescent Protein

FBS: Fetal Bovine Serum

HDACs: Histone Deacetylases

HDAC11: Histone Deacetylase 11

HDAC11KO: HDAC11 Knock-out

IL-1b: Interleukin-1 beta

IL-6: Interleukin-6

IP: Intraperitoneally

LPS: Lipopolysaccharides

mABs: Monoclonal Antibodies

MDSCs: Myeloid-derived Suppressor Cells

MGAL: Murine Genetic Analysis Laboratory

MIP2: Macrophage Inflammatory Protein 2-alpha (MIP2-a)

MPO: Myeloperoxidase

mRNA: Messenger RNA

34 NETs: Neutrophil Extracellular Traps

PBS: Phosphate Buffered Saline

PNs: Peritoneal Neutrophils qRT-PCR: Quantitative Reverse Transcriptase-polymerase Chain Reaction

RACE: Rapid Amplification of cDNA Ends

RFP: Red Fluorescent Protein

TNF-alpha: Tumor Necrosis Factor Alpha

WT: Wild-type

2.2 Abstract

Epigenetic changes in chromatin structure have been recently associated with the deregulated expression of critical genes in normal and malignant processes.

HDAC11, the newest member of the histone deacetylase family of enzymes, functions as a negative regulator of IL-10 expression in antigen presenting cells (APCs) as previously described by our lab. However, at the present time its role in other hematopoietic cells, specifically in neutrophils, has not been fully explored. In this report for the first time we present a novel physiological role for HDAC11, as a multifaceted regulator of neutrophils. Thus far, we have been able to demonstrate a lineage- restricted over-expression of HDAC11 in neutrophils and committed neutrophil precursors (promyelocytes). Additionally, we show that HDAC11 appears to associate

35 with the transcription machinery, possibly regulating the expression of inflammatory and

migratory genes in neutrophils. Given the prevalence of neutrophils in the peripheral

circulation, and their central role in the first line of defense, our results highlight a unique

and novel role for HDAC11. Considering the emergence of new selective HDAC11

inhibitors, we believe that our findings will have significant implications in wide range of

diseases spanning malignancies, autoimmunity, and inflammation.

2.3 Introduction

In humans, the predominant circulating leukocytes—neutrophils, are known to be

produced at immense numbers, through sequences of increasingly differentiated

precursor myeloid progenitors in the bone marrow (BM), before entering the

bloodstream and account for 50-70% of the entire circulating population [114-117].

During granulopoiesis neutrophils are produced at the rate of 1 x 1011 each day with a

significant increase within hours during infections [118], and in patients with various

cancer [119, 120] and therefore more than half of the BM is devoted to the production of

these cells at steady state [121]. Neutrophils play a pivotal and well-defined role in the host defense where they eradicate invading microorganisms [122], and even though they have been labeled short-lived, new in vivo deuterium labeling analysis has revealed that these cells may have a circulatory life span of up to 5 days [123].

Moreover, it is known that neutrophils can influence the immune response by way of communicating with dendritic cells (DCs), macrophages [124, 125] as well as B cells

[126] and T cells [127]. In fact an accumulating series of evidence also suggests that

36 neutrophils have the potential to gain phenotypic as well as functional properties

classically assigned to APCs [128, 129], and in the presence of cancer appose tumor

progression [114, 130] conversely given the appropriate signals, regulate tumor growth

[131-134]. These antagonistic populations of neutrophils referred to as N1—tumor inhibiting and N2—tumor promoting, probably exist as a dynamic array of activation states, rather than only two distinct populations [130, 135]. Additionally, neutrophils can form structures called NETs (neutrophils extracellular traps) which are involved in a process called NETosis—a contributor of innate immunity and induced or stimulated by infection, inflammation, trauma, cytokine production, activated platelets, autoantibodies and pathogens [136-140]. In recent years, NETosis has been identified as an additional

pathway of programmed cells death [141] during which nuclear chromatin relaxes and

forms fibrous web-like structures composed of DNA and histone associated granular

proteins [138, 142]. Therefore, it is of no surprise that these cells can also damage cells

and tissues of self (host), highlighting the importance of regulating genes functionally

responsible for these pathological occurrences [143, 144]. Regulation of neutrophil

function and differentiation in particular genes involved in the inflammatory responses

and chronic diseases, are mostly regulated at the transcriptional levels [145]; and subsequently an identification of factors involved in these processes would offer significant insight into the molecular mechanisms governing functional outcome. For number of years, regulation of normal and malignant hematopoiesis by epigenetic factors has shown to be an area of significant interest [146, 147]. Epigenetic changes in chromatin structure have been associated with the deregulation of critical genes in normal as well as malignant hematopoiesis [148, 149]. Histone deacetylases (HDACs)

37 alter chromatin by deacetylation of histone tails, resulting in transcriptionally inactive

chromatin [150]. HDAC inhibitors have also been identified to alter cytokine production profile [151], ultimately influencing the fate and expansion of hematopoietic cells [148,

152]. However, presently the exact role of specific HDACs in regulation of

hematopoietic processes is yet to be elucidated. Moreover, it has been demonstrated

that cytokine production by myeloid cells is regulated by changes in the acetylation

status of specific gene promoters [153, 154]. HDAC11 has been described as a negative regulator of IL-10 expression in myeloid cells [80]. Also, it has been shown that

lack of HDAC11 increases the suppressive capacity of myeloid derived suppressor cell

(MDSC) [155]. HDAC11, the most recently identified HDAC, is the sole member of the

Class IV HDAC sub-family [79]. The functional role of HDAC11 remains poorly

characterized. Initially it was believed that HDAC11 had a limited tissue expression

restricted to kidney, heart, brain, skeletal muscle, and testis [79]; but it has recently

been documented to also be expressed in hematopoietic cells, where it plays an integral

role in the regulation of immune tolerance through its action in antigen presenting cells,

However at the present time, its regulatory role in myeloid differentiation and specifically

neutrophil function is yet to be characterized. In this manuscript, we reveal a previously

unknown role of HDAC11 which may involve the regulation of neutrophil function. Here

we demonstrate that expression of HDAC11 correlates with neutrophil maturation,

migration, and phagocytic function. We also show that HDAC11 may be involved in the

transcriptional machinery of IL-6, TNF-alpha, and CXCR2/CXCL2.

38 2.4 Material and Methods

2.4.1 Mice and cell lines: C57BL/6 wild-type mice were purchased from Charles

River laboratories, Tg-HDAC11-eGFP reporter mice [156] were provided by Nathaniel

Heintz through the Mutant Mouse Regional Centers, and HDAC11KO kindly supplied by

Merck and obtained from Dr. Seto’s lab respectively. All strains of mice were housed in

the same designated room at the animal facility (Stabile Research-Moffitt Cancer

Center), were kept in pathogen-free condition, and handled in accordance with the

requirements of the Guideline for Animal Experiments. HL60 acute promyelocytic

leukemia (APL) cell line was purchased from American Type Culture Collection (ATCC)

and cultured and maintained in RPMI with 10% FBS, at 5%CO2 and 37°C. Aging

HDAC11KO and C57BL/6 wild-type mice were housed in the same room, under

identical conditions (mentioned above) for 18 months (n=5/group and strain).

2.4.2 Quantitative Reverse Transcriptase-Polymerase Chain Reaction (qRT-

PCR): Total RNA was prepared from centrifugally pelleted and pre-sorted cells (RNeasy mini columns and RNAse free DNAse, Qiagen, Valencia, CA). cDNA was prepared using iScript cDNA Synthesis Kit (Bio-Rad) and qRT-PCR reactions were conducted using the SYBR green two-step qRT-PCR (Bio-Rad) with transcript-specific primers

(Supplied upon request) and cDNA from samples as templates. qRT-PCR amplification reactions were resolved on CFX iCycler (Bio-Rad) and fold changes were quantified (2 -

 C t ) [157]. Primers used TNF-alpha Forward (CCGATGGGTTGTACCTTGTC)

39 Reverse (AGATAGCAAATCGGCTGACG), IL-6 Forward

CCGGAGAGGAGACTTCACAG) Reverse (TCCACGTTTCCCAGAGAAC), 18s Forward

(GTAACCCGTTGAACCCCATT) Reverse (CCGTCCAATCGGTAGTAGCG), CXCL2

Forward (TCCAGAGCTTGAGCGTGACG) Reverse (TTCAGGGTCAAGGCAAACTT),

CXCR2 Forward (TCTGCTACGGGTTCACACTG) Reverse

(ACAAGGACGACAGCGAAGAT).

2.4.3 Flow Cytometry: Flow cytometric analysis of bone marrow aspirates was performed using fluorochrome-labeled monoclonal antibodies (mAbs; anti-CD3, -CD4, -

CD8, -CD19, -NK1.1, -CD11b, -Ly6G, -CD45, -CD117, -CD127, -CD11C, -Ly6G)

Becton Dickinson, San Jose, CA and eBiosciences, San Diego, CA) and the vitality dye

4',6-diamidino-2-phenylindole (DAPI, Sigma). Data was acquired on an LSRII cytometer (Beckman Coulter), at least 10,000 events, of the smallest population of interest, were collected using an LSR II (BD) and subsequently analyzed using Kaluza

(BD) and FlowJo software 10.0.07r2 (TreeStar). Annexin V Flow cytometry was performed utilizing BD’s LSRII flow cytometer using purified peritoneal neutrophils from wild-type C57/BL6 and HDAC11KO mice. A FITC conjugated Annexin V antibody and the DNA dye Propidium Iodide were used in conjunction with FACS buffer containing and Ca/Mg. Gates were created for the analysis of Apoptotic, Viable and Dead cells from total cells. Data was collected to a limit of 10,000 events of the population of interest.

40 2.4.4 Immunoblotting: The cells were lysed in a lysis-buffer containing 50 mM

Tris, 280 mM NaCl, HCL PH 8.0, 0.5% Igepal, 5 mM MgCl2, 10% glycerol and 10X

protease inhibitor (Roche), phosphatase inhibitor (Santa Cruz Biotechnology). Next ,

lysates were sonicated for 8 min (on ice---for 2 cycles of 30s on, 30s rest) and then mixed with 4× gel loading buffer and boiled for 10 min. Samples were then resolved on

10% gels and transferred to nitrocellulose membranes. Membranes were blocked with

5% milk-PBS-Tween. Bands were detected by scanning blots with an LI-COR Odyssey imaging system. Anti-GAPDH (68795) was purchased from Sigma Aldrich. Anti-b-actin

(sc-47778) was purchased from Santa Cruz Biotechnology. Anti-HDAC11 (3611P-100)

was purchased from BioVision Incorporated.

2.4.5 Harvesting and in vitro culturing of mouse peritoneal neutrophils:

C57BL/6 wild-type or HDAC11KO mice (8 weeks old, female) were injected

intraperitoneally (ip) with 1 mL sterile 5% Thioglycollate solution (BD). Mice were

euthanized and peritoneal cavity was flushed immediately. The cavity was flushed with

10 mL ice-cold RPMI-1640 medium (HyClone). Cells from the initial wash were pelleted

and neutrophils were positively selected using biotin anti-mouse Ly-6G antibody

(127604, Biolegend) and Mouse Biotin Selection Kit (EasySep, Stemcell Technologies).

The peritoneal neutrophils were cultured in 6-well (5 × 106 cell/mL) plates, in complete

RPMI-1640 medium in presence of 10% fetal bovine serum (HyClone) and 1% Penicillin

Streptomycin (Corning), stimulated with 2.5 µg/mL Lipopolysaccharides (LPS) (L2880,

Sigma). The purity of the peritoneal neutrophils was about 90% after selection.

41 2.4.6 Migration assay analysis: Medium (25 µL) (control Medium) containing serum-free medium or chemokine MIP2 (10 µg/mL) was loaded into the lower chambers of Transwell system. 50 µL of cells suspension (1×106/mL or 2×106 /mL) in 10 % FBS

RPMI 1640 medium was added to the upper compartment of the chemotaxis chamber.

The two compartments were separated by a 5-µM pore size polycarbonate filter.

Spontaneous migration was determined as the movement of cells toward the control

medium.

2.4.7 Chromatin Immunoprecipitation: Mouse peritoneal neutrophils (PNs)

(3×107) were cross-linked with 1% formaldehyde (F8775-25ML, Sigma) for 10 minutes

at room temperature. Then the cross-linking was stopped by 0.125 M glycine for 10

minutes at room temperature. The cells were washed twice with PBS and proceed with

sample preparation. The cell pellet was lysed by 1 mL of lyse buffer (50 mM HEPES pH

7.8, 3 mM MgCl2, 20 mM KCl, 0.25% Triton X-100, 0.5% NP-40) on ice for 10 minutes.

The cell lysate was transferred to a “Dounce Homogenizer” and 25 strokes were applied using the loose pestle A. The cell pellet was washed once with 1 mL wash buffer (10 mM Tris-HCl pH 8.0, 1 mM EDTA, 200 mM NaCl). The pellet was resuspended in 600 uL sonication buffer (50 mM HEPES pH 7.8, 140 mM NaCl, 1 mM EDTA, 0.5% SDS,

1% Triton X-100). The samples were sonicated and centrifuged for 10 minutes. The lysate was diluted at 1:10 to dilution buffer I (50 mM HEPES pH 7.8, 140 mM NaCl,

1mM EDTA, 1% Triton X-100), pre-cleared using Protein A Agarose beads (16-156,

Millipore), incubated with rabbit anti-HDAC-11 antibody cocktail (3611P, BioVision and

H4539-200UL, Sigma) on a rotator at 4°C overnight. Biotin-conjugated normal rabbit

IgG-B (SC-2763, Santa Cruz Biotechnology) was added at the same amount of rabbit

42 anti-HDAC-11 antibody cocktail in parallel of each sample and incubated on a rotator at

4°C overnight. Then 50 µL protein A Agarose beads was applied to each sample and incubated at 4°C for 4 hours. The beads were then washed twice each with dilution buffer I, dilution buffer II (50 mM HEPES pH 7.8, 500 mM NaCl, 1 mM EDTA, 1% Triton

X-100), LiCL buffer (20 mM Tris-HCl pH 8.0, 250 mM LiCl, 1 mM EDTA, 0.5% Triton X-

100), and TE buffer (10 mM Tris-HCl pH 8.0, 1 mM EDTA). Then the beads were eluted

in 100 µl elution buffer (50 mM Tris-HCl pH 8.0, 1 mM EDTA, 1% SDS) and incubated

at 65°C for 15 minutes and the immunocomplexes were collected in the soluble fraction.

NaCl was added to a final concentration 200 mM and the crosslinking was reverted by

incubating at 65°C for overnight. Then RNAse A (158922, Qiagen) was added to 20

µg/mL to each sample and incubated at 37°C for 30 minutes. Proteinase K (EO0491,

Thermo Scientific) was then added at 100 µg/mL and incubated at 42°C for 2 hours.

DNA was purified by using QIAquick PCR Purification Kit (28104, Qiagen) and purified

DNA was eluted in 100 µL TE buffer for analysis by qRT-PCR. 2 µL of DNA was used

for each qRT-PCR reaction. The following PCR primers were used: TNF alpha Forward

(CTCGGAAAACTTCCTTGGTG), TNF alpha Reverse

(CGATGGAGAAGAAACCGAGA), IL-6 Forward (TCTGCAGAGTGAAGAAGCTGA), IL6

Reverse (GATTCCAGGCTGAAAGTAGGC), CXCL2 (MIP-2) Forward

(GGCTAGAACTGAGGGCTAC), CXCL2 (MIP-2) Reverse

(ACATCCATTCTTGTCCCACTG), CXCR2 Forward (CCTCTGACTCCCACACATCT),

CXCR2 Reverse (GCTTGCTGTGCTTTTGGTTT).

2.4.8 Phagocytosis assay: Upon euthanasia, 1mL of blood was obtained from an experimental mouse via cardiac puncture with a heparinized syringe. Blood was

43 centrifuged at 2,000 RPM for 2 minutes in a microcentrifuge tube and Sera was

aspirated and added to a single tube of lyophilized pHrodo Red E. coli BioParticles (Life

technologies). The BioParticles were vortexed and then incubated for 30 minutes in a

37ºC water bath for optimal opsoninization. BioParticles were centrifuged at 2,000 RPM

for 2 minutes and washed twice with PBS (pH 7.4) w/o Ca and Mg and resuspended in

2 mL of RPMI with 10% FBS. Purified neutrophils were resuspended at 1 x 106 cells per

mL and plated at 100 µL per well, in quadruplicate, in a 96 well flat bottom TC treated

plate per mouse. BioParticles were added to the plate immediately before being placed

into Incucyte High Throughput Live Cell Microscope System for detection of red

fluorescent protein (RFP) expression.

2.4.9 Incucyte real-time microscopy analysis: Cells in a 96 well plate were

imaged through a 10× objective lens at 5 minute intervals for 3 hours using the Incucyte

Zoom HD live cell imaging system (Essen Bioscience, Michigan, USA). The system is

located in a 37ºC /5% CO2 cell culture incubator to maintain proper incubation

conditions. Analysis was performed using the default red fluorescence processing

definition within the Incucyte Zoom 2014B software. The number of red cells and

confluency of red fluorescence was extracted from the images and export the excel

format.

2.4.10 Cytometric Bead Array: This assay involves measurement of secreted cytokines in the media by flow cytometry. Briefly, 100 µL of supernatant from cells

stimulated with or without LPS were collected as specific time-points and IL-6 and TNF-

alpha levels were measured following manufactures recommended protocol CBA kitTM;

(BD Biosciences, San Jose, Ca).

44 2.4.11 Sepsis model: C57BL/6 wild-type and HDAC11KO mice were injected

with 15 mg/Kg of LPS (L4391-1MG Sigma) via tail vein (TV) in 100 µL volume and

observed for signs of sepsis (abnormal posture/positioning, partial paralysis, failure to

respond to stimuli, head tilt, circling, impaired locomotion, non-purposeful movement,

hypoactivity, hyperactivity, restlessness, self-trauma, aggressiveness, isolation from

cage mates, shallow, rapid and/or labored breathing, cyanosis, piloerection, matted hair

coat, lack of inquisitiveness, hunched posture and/or vocalizations) 3 times daily.

2.4.12 RACE analysis: This experiment was out-sourced to murine genetic analysis laboratory, mouse biology program at University of California, Davis. Detailed protocol will be provided upon request.

2.4.13 Statistical Analysis: Unless otherwise specified, the statistical significance between values was determined by student’s t test. Data were expressed as the mean ± SD. Probability values of p ≤ 0.05 were considered significant.

2.5 Results

2.5.1 HDAC11 is differentially expressed in various stages of myeloid

differentiation

It has previously been reported that HDAC11 is a negative regulator of IL-10 production in myeloid cells. Here, we examined the expression of HDAC11 in myelopoiesis. To follow the dynamic changes in HDAC11 transcriptional activation, we utilized HDAC11 promoter-driven eGFP reporter mice (Tg-HDAC11-eGFP) [156], where

45 eGFP expression is indicative of HDAC11 transcription. Using multiparameter flow

cytometric analysis, as seen in Figure 2.1A, in the bone marrow compartment, we were

able to determine that expression of HDAC11 is markedly increased in promyelocytes

when compared to earlier progenitors and further amplified in the neutrophils when

compared to myeloblasts. Interestingly, expression eGFP and consequently of

HDAC11 in monocytes was negligible when compared to promyelocytes and

neutrophils (Figure 2.1A). The lymphocytes also show a moderate expression of

HDAC11 transcript. Expression of HDAC11 protein was confirmed by immunoblotting

analysis in flow-sorted monocytes, promyelocytes and neutrophils (Figure 2.1B top

panel). Quantitative mRNA analysis confirmed our findings by demonstrating an

increase in the expression HDAC11 message (fold changes are normalized to the 18s

house-keeping gene for each sample) and further validated the Tg-HDAC11-eGFP

reporter mouse model (Figure 2.1B lower panel) used in our experiments. Additionally,

we further validated these results by quantifying the mRNA expression of eGFP in the

same samples and were able to demonstrate that expression of HDAC11 is

concomitant with the expression of eGFP message (Figure 2.1C). To ensure that our

data was not an artifact of the eGFP reporter mouse model, we flow-sorted monocytes, promyelocytes, neutrophils, and lymphocytes from a C57BL/6 wild-type mouse and examined the expression of HDAC11 message (Figure 2.1D). The results confirmed our

previous findings in the Tg-HDAC11-eGFP reporter mouse and consequently the

neutrophil population expressed significantly higher levels of HDAC11 when compared

to the rest of the sub-populations. Next, we purchased source leukocytes (OneBlood,

Florida Blood bank) collected from volunteer normal donors and isolated (flow-sorted

46 cell populations) monocytes and neutrophils. Quantification of HDAC11 expression

observed in this figure show parallel results mirroring what we had previously seen in

our murine models where neutrophils had higher expression of HDAC11 message

(Figure 2.1E lower panel). Immunoblotting analysis confirmed the expression of

HDAC11 in the neutrophils when compared to the monocyte population (Figure 2.1E top

panel).

Figure 2.1 HDAC11 message is differentially expressed in various stages of myelopoiesis. (A) Dynamic visualization of HDAC11 message using HDAC11-eGFP transgenic mouse in myeloid compartments. Bone marrow was extracted from Tg- HDAC11-eGFP (As well as CD57B/6 mice as control- non-eGFP expressing cells; gray histogram markings) and cells were labeled (as indicated above) with specific cell surface markers for identification of each population. HDAC11 expression was determined by flow cytometry analysis of eGFP reporter gene expression, where the

47 expression of eGFP protein corresponds to the activation of HDAC11 transcriptional machinery. (Representative presentation from 2 individual experiments) (B) Monocytes, promyelocytes, and neutrophils from Tg-HDAC11-eGFP mouse bone marrow cells were sorted using FACS-Aria (BD) device with 99% purity. Cells were lysed using TriZoL reagent (Invitrogen) as well as RIPA buffer (Cell Signaling) and RNA as well as protein was extracted respectively. Immunoblotting was performed using 10% SDS gel and image was resolved using Dura ECL reagent (Pierce) (Top image) and qRT-PCR analysis was performed using HDAC11 primers (bottom graph). Sorted cell populations were also morphologically confirmed (Lower panel). (C) Co-expression of HDAC11 message and eGFP message was confirmed by qRT-PCR using eGFP primers. (Error bars = S.E.M; data representative of 3 individual experiments n=3) (D) Monocytes, promyelocytes, neutrophils, B-cells, and T cells were sorted from C57BL/6 wild-type mouse bone marrow cells using FACS-Aria (BD) device with 98% purity. Cells were lysed using TriZoL reagent (Invitrogen) and RNA was extracted. qRT-PCR analysis was performed using HDAC11 primer. This was done to confirm HDAC11 expression profile in a non-transgenic setting. (Error bars = S.E.M; data representative figure from 3 individual experiments n=3). (E) Four normal human donor peripheral blood source leukocyte samples (Purchased from OneBlood Florida) were sorted for monocytic and neutrophylic populations using FACS-Aria (BD) device with 96% purity and then examined for the expression of HDAC11 message using qRT-PCR analysis and the fold change between neutrophils and monocytes were normalized to the lowest expressing monocytic population. (Error bars = S.E.M, n=4) Note: In figures B, C, D, and E sub- populations were compared and normalized to monocytes (Since they have the lowest expression of HDAC11).

2.5.2 Expression of HDAC11 is significantly increased in leukemic cells

upon differentiation

Utilizing HL60 acute promyelocytic leukemia (APL) cell line model and upon in vitro maturation and differentiation of these cells using all trans-retinoic acid (ATRA) stimulation, we observed an increase in the expression of HDAC11 message (Figure

2.2A bottom) and protein (Figure 2.2A top) as demonstrated. These differentiated HL60 cells also exhibited neutrophil-like segmentation and over-expression of maturation markers such as CD11b, and myeloperoxidase (MPO) (Figure 2.2B).

48 Figure 2.2 Expression of HDAC11 in acute promyelocytic leukemia samples (APL) and functional consequences of its manipulation in this model. (A) HL60 cells were treated with All Trans Retinoic Acid (ATRA) for 72hrs and the expression of HDAC11 message was measured by qRT-PCR and the differentiation of HL60 cells to granulocytic-like cells were examined by morphological analysis (Left) Error bars = S.E.M; (Representative figure from 3 individual experiments) . (B) The differentiation of HL60 cells to granulocytic-like cells were examined using Myeloperoxidase (MPO-a marker for cell’s gain of granularity) (Top) as well as CD11b expression (Bottom). (Representative figure from 3 individual experiments).

2.5.3 Lack of HDAC11 markedly increases the expression of pro-

inflammatory cytokines

HDAC11 is restricted mainly to the nucleus, and its expression appears to be tissue specific with higher expression in brain, heart, testis, and skeletal muscle [79],

however its precise function in myeloid compartment and specifically in the neutrophils

49 is yet to be elucidated. In the previous experiments, we utilized germ-line HDAC11

knock-out mice (HDAC11KO) (Merck Pharmaceutical). HDAC11KO (C57BL/6

background) mice were developed using Lox/CRE technology to remove a floxed exon

3, a portion of the histone deacetylase catalytic region. In our Figure 2.3A, we

demonstrate our method of positively genotyping the HDAC11KO mice. Since these

mice will still transcribe a truncated mRNA sequence (excluding exon3), and to further

validate this mouse model, we designed a special HDAC11 primer set that was within

the excised exon3 region of HDAC11 gene. As seen in supplementary figure,

HDAC11KO mice have no amplified HDAC11 amplicons demonstrated by qRT-PCR

analysis when compared to C57BL/6 wild-type counterparts (Figure 2.3B). Also,

immunoblotting analysis suggested that the expression of HDAC11 is absent in cells

isolated from HDAC11KO mice (Figure 2.3C). Taking a step further, we sent samples

from two C57BL/6 wild-type and two HDACK11KO mice to Murine Genetic Analysis

Laboratory (MGAL), Mouse Biology Program at University of California, Davis for Rapid

Amplification of cDNA ends (RACE-Seq). The data confirmed that the HDAC11KO mice are devoid of exon3 on the HDAC11 gene sequence.

Recently, it has been demonstrated that epigenetic factors such as HDACs have

been known to affect regulation of genes involved in inflammatory responses [158].

Subsequently, we decided to investigate whether lack of HDAC11 had any functional

consequences in the neutrophil population. To do this, we isolated peritoneal

neutrophils (PNs) from both HDAC11KO as well as C57BL/6 wild-type mice; 18 hours

post 5% thioglycollate injection. In vitro, cells were treated with 2.5 µg/mL of

50

Figure 2.3 (A) Schematic representation of HDAC11KO mouse development and genotyping methods for proper identification of mouse colony. (B) qRT-PCR analysis of HDAC11 expression (using exon3-designed primers) in brain and spleen of C57BL/6 wild-type and HDAC 11KO mice (n=3). (C) Expression of HDAC11 protein in brain and testes of C57BL/6 wild-type and HDAC 11KO mouse. lipopolysaccharide (LPS) for 2 and 4 hours and the samples were analyzed for TNF-

alpha and IL-6 message and protein expression. Results revealed that in the absence of

HDAC11, neutrophils became more inflammatory as seen by the mRNA expression

level of TNF-alpha and IL-6 at every time-point (Figure 2.4A). These findings were

further confirmed by cytometric bead array (CBA) protein analysis where both cytokines

51 showed higher level of expression when compared to the C57BL/6 wild-type

counterparts (Figure 2.4B). We were also interested to see whether lack of functional

HDAC11 changed the normal distribution of hematological cells in the peripheral blood.

Briefly we collected peripheral blood from HDAC11KO as well as their normal

counterparts C57BL/6 wild-type mice (sub-mandibular blood collection) and performed a

complete blood count (CBC) analysis. Surprisingly, both cohorts—HDAC11KO as well

as the C57BL/6 wild-type control mice had relatively similar monocytic and granulocytic

blood count profile (Figure 2.4C). To our knowledge thus far, there has been no report of HDAC11 binding directly to DNA and consequently, there are no direct downstream genes that can be signature indicators of this interaction. In experiments for this section we also interrogated the possibility of HDAC11 being involved in transcriptional regulation of these molecules. Briefly, PNs isolated from C57BL/6 wild-type mice were

treated with 2.5 µg/mL of LPS for 3.5 hours (the concentration and the time point for

these experiments were determined by dose titration and time course analysis in

preceding experiments) and then the cells were processed for Chromatin

immunoprecipitation (ChIP) assay. In Figure 2.4D, ChIP data suggests that HDAC11

may be recruited to the TNF-alpha and IL-6 transcriptional complex. To demonstrate

that this observation was specific to HDAC11 being possibly recruited to the promoter

region of TNF-alpha and IL-6, we performed a similar ChIP analysis in mice using

HDAC11KO PNs which showed no recruitment of HDAC11 to the promoter regions of

TNF-alpha and IL-6 interpreted by no change in the enrichment ratio when comparing

LPS stimulated samples to the non-stimulated controls (Figure 2.4E).

52 Figure 2.4 Phenotypic consequences of HDAC11 deficient peritoneal neutrophils. (A) Peritoneal neutrophils (PNs) from C57BL/6 wild-type and HDAC11KO mice were collected post thyoglycolate injection (5% at 18hrs). The cells were treated with 2.5 µg/mL of LPS for 2, and 4hrs. Expression of inflammatory genes TNF-alpha and IL-6 were measured by qRT-PCR. (B) Protein concentrations for TNF-alpha and IL-6 were measured by cytometric bead array (CBA) analysis. (C) Complete blood count from C57BL/6 wild-type and HDAC11KO (n=6 per group). (D) The recruitment of HDAC11 protein to chromatin fragments of TNF-alpha and IL-6 promoters were analyzed using the PfaffI method [157] and are presented relative to input before immunoprecipitation and the enrichment ratio was normalized to the IgG control (PNs isolated from C57BL/6 wild-type) (E) PNs isolated from HDAC11KO mice were used in another ChIP assay as negative control. (NT = No LPS incubation). (Error bars = S.E.M; data presented for each graph is a representative figure from 2 individual experiments).

2.5.4 Neutrophils isolated from mice lacking HDAC11 demonstrate much

higher migratory and phagocytic capacity

53 The hallmark of neutrophils in the innate immune system is their capacity to

migrate to the site of tissue injury and/or infection. In order to interrogate migratory

capacity of neutrophils lacking HDAC11, we isolated peritoneal neutrophils (as

mentioned in Figure 2.4) and performed a migration assay. Briefly, in this assay

isolated neutrophils were loaded in the upper compartment of the chemotaxis chamber,

and the serum-free media or chemokine CXCL2-(MIP-2—IL-8 homologue) (10 µg/mL) was loaded into the lower chambers of a transwell system, and the assay was concluded by detection and calculation of migratory neutrophils. The data suggests that neutrophils isolated from the HDAC11KO mice have a significantly higher migratory capacity towards a bait chemokine MIP-2 when compared to the wild-type counterpart

(Figure 2.5A). Next we compared the expression of migratory receptor/chemokines such as CXCR2 (Homologue of IL-8 receptor beta) /CXCL2, in HDAC11KO and

C57BL/6 wild-type mice. In Figure 2.5B we demonstrate a higher endogenous expression of mRNA for these molecules in neutrophils lacking HDAC11. Flow cytometry analysis confirmed a significant increase in the protein level of CXCR2 on

HDAC11KO neutrophils when compared to the C57B/6 wild-type mice (Figure 2.5C), however the changes in the CXCL2 protein levels were not significant (data not shown).

Furthermore we assessed the phagocytic ability of C57BL/6 wild-type versus

HDAC11KO mouse PNs. Briefly, equal number of PNs from C57BL/6 wild-type and

HDAC11KO mice were co-cultured with E. coli BioParticles loaded with a pH sensitive fluorescent dye. Using live microscopy, we were able to (in real-time) monitor phagocytic capacity of PNs from each mice. Data ascertained from these experiments demonstrated that PNs from the HDAC11KO mice were functionally more potent

54 Figure 2.5 Enhanced migratory and phagocytic capacity of HDAC11KO neutrophils. (A) Migration assay analysis between C57BL/6 wild-type and HDAC11KO PNs utilizing the Transwell system and 2 x 106 per sample. Expression of migratory genes were analyzed in PNs isolated from the WT and HDAC11KO mice using qRT-PCR analysis (Data generated from 4 individual experiments) **p-value < 0.01, * p-value < 0.05. (B) mRNA expression of CXCL2 and CXCR2 were analyzed in both C57BL/6 wild-type and HDAC11KO mice at steady state. (Representative figure from 2 individual experiments). (C) Expression of surface CXCR2 protein on neutrophils from HDAC11KO vs C47BL/6 wild-type mice. (D) Phagocytic ability of PNs isolated from both C57BL/6 wild-type and HDAC11KO mice were analyzed in the presence of E. coli Bio-Particles loaded with a pH sensitive fluorescent dye and analyzed as a measure of cell engulfment and lysis in real-time (RFP measurement by microscopy). (Error bars = S.E.M; statistical analysis was done using two-way ANOVA **p-value < 0.01, * p-value < 0.05. Data presented is a representative figure from 2 individual experiments). (E) The recruitment of HDAC11

55 protein to chromatin fragments of CXCL2 and CXCR2 promoters was analyzed in the presence and absence of LPS stimulation. (F) PNs from HDAC11KO mice were used in another ChIP assay as negative control. The values obtained for these ChIP experiments were analyzed using the PfaffI method [157] and are presented relative to input before immunoprecipitation and the enrichment ratio was normalized to the IgG control. (Error bars = S.E.M; representative figure from 2 individual experiments, three animals were used for each experiment).

(Figure 2.5D). Given our findings discussed in the above experiments, it was deemed necessary to determine if there HDAC11 was being recruited to the promoters of

CXCR2 and CXCL2. Given our presented data so far, we predict that HDAC11 may be a negative regulator of genes involved in migratory response. To examine this hypothesis, we performed a ChIP analysis. Briefly, PNs from C57BL/6 wild-type mice were treated with 2.5 µg/mL of LPS for 3.5 hours and then the cells were processed for

ChIP assay. The data indicates that there is recruitment of HDAC11 to the promoter regions of CXCR2 and CXCL2 (Figure 2.5E). To demonstrate that this observation was specific to HDAC11, we performed a similar ChIP analysis using HDAC11KO PNs which showed no change in the enrichment ratio when comparing LPS stimulated samples to the non-stimulated controls. The observed recruitment of HDAC11 to the promoter regions is not defined as a direct biding of HDAC11 to the promoter regions however, the interrogation of this possibility requires additional and in depth promoter binding analysis in the future.

2.5.5 HDAC11KO mice are more susceptible to LPS induced sepsis when

compared to C57BL/6 wild-type counterpart

Sepsis in humans, defined as severe inflammation in the presence of infection, is a common syndrome that kills thousands of patients each year [159]. To study this

56 physiological response, murine models have been established and validated. For instance, in intoxication models mice are inoculated with proinflammatory compounds(noninfections) such as lipopolisaccharides (LPS) [160]. Of note, mice are extremely resilient to most types of inflammation when compared to humans, but at high doses of LPS within the range of 1-25 mg/kg (1000-10000 X the dose that will cause septic shock in humans), mice experience severe inflammatory response and eventually succumb to sepsis [161, 162]. Given our observations thus far, it appears that

HDAC11KO PNs, at steady state, have a higher innate inflammatory nature when compared to the C57BL/6 wild-type mice. Therefore, we sought to investigate whether there was a difference between the time of inflammatory onset, leading to eventual sepsis and death (post LPS inoculation), when comparing HDAC11KO and C57BL/6 wild-type mice. Briefly, a cohort of HDAC11KO and age matched C57BL/6 wild-type mice (n=8 in each group), were injected with LPS at 15 mg/kg via tail vein. Mice were monitored 3 times in 24hrs. As seen in Figure 2.6, although all mice post LPS inoculation showed signs of fatigue and sluggish movements, HDAC11KO mice expired within the first 48hrs of the experiment while the remaining C57BL/6 wild-type mice fully recovered and were ultimately euthanized at 72hr time-point to mark the termination of the experiment.

57 Figure 2.6 Septic shock experiment comparing HDAC11KO mice and C56BL/6 mice. A cohort (n=8) of HDAC11KO and C57BL/6 mice were injected with 15 mg/kg of LPS via tail vein. Survival graph representing both groups in time-line up to 72 hours. (Data presented is a representative figure from 2 individual experiments).

2.5.6 HDAC11KO mice show bone marrow hypercellularity with

granulocytic expansion and splenomegaly due to increased extramedullary

hematopoiesis

58 Emergency granulopoiesis is defined as a well-coordinated de novo production of

neutrophils due to increased myeloid progenitor cells proliferating in the bone marrow,

signaled by and during severe infection. The ultimate goal of this physiological

occurrence is to increase the neutrophil output during an innate immune response. In

order to see whether HDAC11KO mice had a more robust production of neutrophils, we

injected (i.p.) a cohort of HDAC11KO and C57BL/6 wild-type mice with Freund’s

complete adjuvant (Thermo Scientific) and assessed the expression of granular cells by

flow cytometry. Flow cytometery analysis revealed a moderate increase of granular

cells in the spleen of the HDAC11KO mice when compared to the C57BL/6 wild-type mice however this difference was not significant. Immunohistochemisty (IHC) and H&E staining of bone marrow cells from the same mice demonstrated extensive hypercelullarity which did not allow decisive identification and quantification of granular cells (data not shown). However, when we compared spleen sections from aging

(18months old) HDAC11KO and C57BL/6 wild-type mice, we observed that the

HDAC11KO spleens had increased extramedullary hematopoiesis resulting in an expanded red pulp (figure 2.7A). In addition, sections from femoral bones revealed a marked bone marrow hypercellularity on aging HDAC11KO mice, mostly due to an expansion of maturing neutrophils (figure 2.7B).

59 Figure 2.7 Observation of splenomegaly as well as Hypercellularity with granulocytic expansion in BM of HDAC11KO mice. (A) Representative sections of spleens (H&E stain) harvested from aged C57BL/6 wild-type (top) and HDAC11 KO (bottom) mice showing a marked expansion of the red pulp (left, x40), due to replacement of the mostly lymphocytic red pulp cellularity observed on C57BL/6 wild-type mice with mostly trilinegae extramedullary hematopoiesis on the HDAC11KO mice (right, x400). (B) Representative sections of femurs (H&E stain) harvested from aged C57BL/6 wild-type (top) and HDAC11 KO (bottom) mice showing a marked increase in the bone marrow cellularity on HDAC11KO mice compared to WT mice (left, x20), mostly due to an expansion on maturing neutrophils (right, x200). (n=5 per group)

60 2.6 Discussion

In myelopoiesis, lineage-specific transcription factors C/EBPα, C/EBPε, PU.1,

and AML1 cooperatively interact with specific DNA sequence response elements to

initiate transcription of genes involved in differentiation [121, 163-165]. More recently, epigenetic mechanisms such as chromatin modification by acetylation/deacetylation of histone tails have been shown to contribute to the regulation of gene expression and determination of cell population specificity [166, 167]. Conversely, it has been demonstrated that the deregulation of epigenetic mechanisms cause genetic alterations which consequently leads to manifestation of diseases such as cancer and autoimmunity [102, 168]. More specifically, epigenetic modulations have been reports in number of cellular processes involved in neutrophil development and functions

(reviewed in [147]) including NETosis [169].

In this report, the interrogation of the transcriptional activity of HDAC11 in myeloid and lymphoid cells, utilizing a HDAC11 promoter-driven eGFP reporter mouse model, combined with utilization of lineage-specific markers with multiparametric flow cytometry analysis demonstrated a significant over-expression of HDAC11 in neutrophils, when compared to monocytes (Figure 2.1A). Moreover, analysis of hematopoietic cells isolated from the bone marrow of the Tg-HDAC11-eGFP mice revealed an over-expression of HDAC11 at the promyelocyte stage of neutrophil differentiation and with a significant increase in the neutrophils. Monocytes showed low to undetectable expression of HDAC11 (Figure 2.1B & C). Additionally, our results utilizing flow-sorted leukocyte subpopulations from C57BL/6 wild-type mouse bone marrow, further confirmed and demonstrated a higher level of HDAC11 expression in

61 promyelocytes and neutrophils compared to monocytes and lymphoid subsets (Figure

2.1D). Equally, flow-sorted monocytes and neutrophils from normal human donors’ source leukocytes showed higher levels of HDAC11 expression in neutrophils, as compared to monocyte population (Figure 2.1E). These findings are of interest because they suggest that HDAC11 may be a factor during myelopoiesis and consequently play a role in the fate of neutrophils. Epigenetic mechanisms such as histone modifications play a key role in the control of multiple normal biological processes including hematopoiesis as well as alterations leading to many diseases such as autoimmunity and cancer [170, 171]. Understanding the role of each specific HDAC in the context of the immune system, in different malignancies, will facilitate selective cancer immunotherapeutic modalities. Therefore we further examined HDAC11 in a cancer model to determine whether this lineage-specific overexpression also applied to malignancies using HL60 acute promyelocytic leukemia (APL) human cell line. Our findings supported what we had seen in normal processes. Upon in vitro ATRA induced differentiation and maturation of APL cells, expression of HDAC11 is increased exponentially (Figure 2.2A). Concurrently the expression MPO and CD11b (markers of granularization and) were increased (Figure 2.2B) during maturation of APL cells. The exploration of the physiologic role of HDAC11 overexpression in neutrophils, utilizing a model of germline-HDAC11KO mice demonstrated that purified neutrophils lacking

HDAC11 displayed an apparent overproduction of TNF-alpha and IL-6 upon stimulation with LPS both at message and protein levels, as compared to their C57BL/6 wild-type counterparts (Figure 2.4A & B). Our assessment of these collective data suggests that

HDAC11 may be playing the role of a check-point molecule; where it possibly controls

62 the activation of neutrophils. Moreover, subsequent data suggests that HDAC11 may

be associated with the transcriptional machinery of inflammatory molecules TNF-alpha and IL-6 as seen by HDAC11-chromatin binding which was demonstrated by ChIP analysis (Figure 2.4D & E). Recently, Stammler, et al reported that HDAC11 inhibition increases IL-1b in dendritic cells and macrophages through promoting nonanonical caspase-8-dependent pathway [172]. In our experiments, ChIP analysis revealed that

HDAC11 –chromatin binding is distinctive to TNF-alpha and IL-6 and no binding was observed for IL-1b (IL-1b data not sown). Markedly, migration assay analysis also demonstrated that neutrophils isolated from HDAC11KO mice exhibit a significantly higher migratory rate as well as increased phagocytic activity when compared to the

C57BL/6 wild-type mice (Figure 2.5A). This observation is highlighted by qRT-PCR analysis that showed HDAC11KO mice demonstrate an endogenous over-expression of

CXCR2 and CXCL2 genes (Figure 2.5B & C). Moreover, ChIP analysis results suggest that HDAC11 may also be recruited to the transcription complex, regulating the transcription machinery of CXCR2 and CXCL2 (Figure 2.5E & F). Recently published data suggests that HDAC11 has functional network protein association with number of biological processes including gene expression [173], therefore highlighting the possibility of its involvement in regulating transcription which is yet to be determined.

Typically, occurrence of severe sepsis also known as septic shock is fatal from complications of infection, usually caused by dysregulated inflammatory and immune responses. The onset of sepsis is generally due to a robust innate immune response, through enhanced granulopoiesis in the bone marrow and generation of exuberant number of neutrophils, and consequently a massive production of pro-inflammatory

63 cytokines [174]. Sepsis remains a serious health issue and therefore identification of factors contributing to it is of great interest. Thus far, numerous studies have labeled pan-HDAC inhibitors fundamentally as negative regulators of gene expression for acute immune receptors and pathways in innate immune cells [175, 176]. However oversimplifying this statement cannot be generally correct since HDAC inhibitors depending on tissue type and time of treatment, can have alternative effects on gene expression. Consequently, numerous investigators are now focusing on selective HDAC inhibitors to narrow the target of interest. In regards to HDAC11, our observations suggest that this HDAC maybe a gatekeeper of inflammatory response in neutrophils. In fact, in a murine sepsis model, we were able to show that HDAC11KO mice succumb to sepsis much faster than the C57BL/6 wild-type counterparts (Figure 2.6) suggesting the

possible presence of pre-primed neutrophils in HDAC11KO mice. Additionally, CellTiter-

Blue® viability assay demonstrated a modest decrease in the number of neutrophils

(isolated from the BM) extracted from HDAC11KO mice in the absence of stimulation

when compared to the C57BL/6 wild-type counterpart however, a significant decrease in

the viability of neutrophils isolated from the HDAC11KO mice was observed when these

cells were stimulated with GM-CSF, compared to C57BL/6 wild-type counterparts

(Figure 2.8). Moreover, in aging HDAC11KO mouse experiment we observed

noticeable increase in the bone marrow cellularity when compared to age-matched

C57BL/6 wild-type controls mice, which is mostly due to an expansion of maturing neutrophils (Figure 2.7). This is indicative of what we have demonstrated—signifying the likelihood of an essential role for HDAC11 in neutrophil biology.

64

Figure 2.8 BM cells from the femurs of age-matched WT (C57BL/6) and/or HDAC11KO (C57BL/6) mice (n=4/ mouse strain) were extracted and neutrophils from these samples were isolated using the StemCell™ EasySep magnetic selection kit. Each sample was plated at equal numbers in triplicates in a 96-well plate. Cells were then treated (or not in the control groups) with 50ng/ml of mouse GM-CSF for 9 hours. Next CellTiter-Blue® reagent was added to each well and incubated at 37C for 1 hour following by the measurement of fluorescent signal at 560(20)Ex/590(10)Em using a BioTek Cytation 3 plate reader. (Data is a representative graph from 2 independent experiments).

In recent years, the role of neutrophils has significantly expanded from front-line

combatants of infection to endowment of anti-tumor immunity [177, 178]. In contrast,

recent evidence suggests a novel pro-tumor function for these cells [124, 179],

suggesting complex and opposing roles of neutrophils in a tumor setting. Such

observations continue to highlight the importance of identifying factors that may play

65 essential roles in neutrophil activation and function. In conclusion, our data suggests

that HDAC11 appears to have a dual function in neutrophils biology; 1) HDAC11 is increased as neutrophils differentiate and mature, and 2) decrease in HDAC11 correlates with functional activity of neutrophils. Our studies reveal that HDAC11 plays a

role in neutrophil chemokine and cytokine biology and function. Given the multifaceted

function of neutrophils, the key findings described here will potentially lead to targeted

epigenetic therapies to influence diseases involving neutrophils.

66

Chapter 3: HDAC11 Function as a Transcriptional Regulator in Immature Myeloid

Cells (IMCs) to Myeloid-derived Suppressor Cells (MDSCs) transition

3.1 List of Abbreviation

APCs: Antigen Presenting Cells

ATCC: American Type Culture Collection

C/EBPβ: CCAAT-enhancer-binding protein beta

ChIP: Chromatin Immunoprecipitation

DCs: Dendritic Cells

FBS: Fetal Bovine Serum

HDACs: Histone Deacetylases

HDAC11: Histone Deacetylase 11

HDAC11KO: HDAC11 Knock-out

IL-1b: Interleukin-1 beta

IL-6: Interleukin-6

IMCs: Immature Myeloid Cells

67 iNOS: Inducible Nitric Oxide Synthetase

IP: Intraperitoneally

IV: Intravenous

LPS: Lipopolysaccharides

MDSCs: Myeloid-derived Suppressor Cells mRNA: Messenger RNA

NO: Nitric Oxide

PBS: Phosphate Buffered Saline qRT-PCR: Quantitative Reverse Transcriptase-polymerase Chain Reaction

WT: Wild-type

3.2 Abstract

Myeloid-derived suppressor cells constitute a heterogeneous population of immature myeloid cells derived from bone marrow and possess the ability to negatively regulate both innate and adaptive immunity in the tumor microenvironment in large scale solid tumor settings. Previous work in our lab had demonstrated that MDSCs lacking histone deacetylase 11 (HDAC11), a newly identified member of the HDAC family, revealed an increase in suppressive ability against CD8+ T cell cytotoxicity.

However, the mechanisms of HDAC11 that contribute to the suppressive function of

68 MDSCs remain unclear. In this study, we have evaluated a number of factors implicated in MDSCs-mediated immune suppression, such as arginase activity, NO and ROS production. Our data suggest that arginase activity and NO production is significantly higher in HDAC11 knockout MDSCs when compared with C57BL/6 wild type control.

Acquisition of this elevated suppressive function in HDAC11 KO MDSCs is possibly mediated through an up-regulation of transcription factor CCAAT-enhancer-binding protein beta (C/EBPβ). Also, we observed a markedly higher expression of C/EBPβ in

CD11b+ Gr1+ from HDAC11 KO animals under tumor challenge as well as at steady state. Additionally, our data showed that HDAC11 was recruited to the promoter region of C/EBPβ in wild-type MDSCs, suggesting that HDAC11 serves as a negative regulator of C/EBPβ.

3.3 Introduction

In both cancer patients and tumor bearing animals, the presence and progression of tumors often contribute to the development of immune dysfunction which introduces challenges and negative feedbacks to the development of immunotherapeutic strategies. Multiple cell types have been identified to regulate tumor mediated immune evasion and immunosuppression, including regulatory T cells [180], tumor associated macrophages and MDSCs . As one of the major cell populations that infiltrated into the tumor site, MDSCs are believed to involve largely with the unresponsiveness of antigen specific CD8+ T cells in the tumor microenvironment [181].

Recently, two major subsets of MDSCs have been identified based on their morphology

69 and surface markers expression: monocytic MDSC (M-MDSC) and granulocytic MDSC

(G-MDSC, also called polymorphonuclear PMN-MDSC) [35, 36]. In mice, MDSCs are

recognized as cells that express Gr-1+CD11b+ phenotype and further M-MDSCs are

characterized as CD11b+Ly6G-Ly6Chigh while G-MDSCs are characterized as

CD11b+Ly6G+Ly6Clow [36-38]. However, in cancer patients, the phenotype of human

MDSCs remains unclear. So far, human M-MDSCs are defined as

CD11b+CD14+CD33+HLA-DR-/lowCo-receptor-/low and while G-MDSCs as

CD11b+CD15+CD33+Lin-HLA-DR-/low expressing cells [8, 39].

MDSCs display a wide range of mechanisms to restrain the function of antigen

specific and non-specific T lymphocytes in vitro. They express high levels of

immunosuppressive arginase and NO/nitric oxide synthetase (iNOS), as well as

reactive oxygen species (ROS) [38, 42, 162, 182, 183]. A recent study has revealed several transcription factors that are activated by GM-CSF, IL-6 and other cytokines or growth factors, which regulate MDSC differentiation and function. Marigo and colleagues had recently provided new insight demonstrating that the transcription factor

CCAAT-enhancer-binding protein beta (C/EBPβ) plays a crucial role in the differentiation and immunosuppressive function of BM-derived and tumor-induced

MDSCs [31]. Of note, mice lacking C/EBPβ in the bone marrow compartment lose the ability to differentiate from IMCs into pathologically active MDSCs, possibly through a reduction of arginase 1 and Nos2 proteins [31].

A growing knowledge of epigenetic modification has been shown to regulate hematopoiesis under different pathological conditions such as infection and cancer

[146, 184]. HDAC11, the newest member of the HDAC family, was first identified in

70 2002 as a 39 kDa enzyme and the only class IV HDAC [79]. Northern analyses and

real-time PCR data from Gao and colleagues had confirmed a high expression level of

HDAC11 in brain, testis, some types of B-cell lymphomas and leukemias in humans respectively [79]. The functional role of HDAC11 played in the immune system was

unclear until Villagra et al first uncovered that this HDAC serves as a negative regulator

of IL-10 expression in macrophages [80]. Neutrophils lacking HDAC11 displayed a

functionally more active phenotype as well as enhanced migratory and phagocytic

capacity [185]. Moreover, data published by Woods and colleagues showed that down-

regulation of HDAC11 was required for T cell activation. This has further been

confirmed with a hyper-proliferative and responsive phenotype of HDAC11 Knockout T

cells [82]. These studies hinted at a positive feedback of innate and adaptive immune

response in a system devoid of functional HDAC11 protein. Although adoptive transfer of HDAC11KO T cells had significantly delayed tumor progression in a murine syngeneic lymphoma model [82], inoculation of murine thymoma or pancreatic cancer

cells to HDAC11 total KO mice demonstrated enhanced tumor growth kinetics when

compared with wild-type [155], hinting a hidden mechanistic role of HDAC11 in the

immunosuppressive compartments in cancer. Notably, Sahakian had uncovered that in

responding to antigen-specific OVA peptide, IFN-γ production from OT-1 CD8+ T cells

was significantly decreased when co-cultured with HDAC11KO MDSCs when compared

with Wild-type MDSCs [155], indicating that HDAC11KO MDSCs are functionally more

suppressive. However, the mechanism of HDAC11 in MDSCs expansion and function

remains to be elucidated. Here for the first time, we demonstrate a previously unknown

mechanistic role of HDAC11 as a transcriptional regulator of C/EBPβ in MDSCs.

71 Indeed, in the absence of this epigenetic checkpoint of C/EBPβ gene expression, the fully suppressive potential of MDSCs was fully unleashed. A better understanding of this novel role of HDAC11 in myeloid biology will ultimately lead to targeted epigenetic therapies to manipulate the suppressive abilities of these immunoregulatory cells.

3.4 Material and methods

3.4.1 Mice and cell line: C57BL/6 and B6-Lyz2tm1(cre)lfoIJ mice were purchased from The Jackson Laboratory. HDAC11 knockout mice were supplied by Merck

Research Laboratory and obtained from Dr. Seto’s laboratory at The George

Washington University Cancer Center. The HDAC11KO mice were on a C57BL/6 background and were generated by targeted deletion of floxed exon 3 of the HDAC11 gene. HDAC11 myeloid cell conditional knockout (HDAC11 KO LyZ-Cre) mice were generated by breeding B6-Lyz2tm1(cre)lfoIJ transgenic mice with HDAC11 flox/flox mice for greater than 10 generations. The third exon of HDAC11 gene, flanked by LoxP sites, is excised only in myeloid cells expressing the lysozyme 2 gene (Lyz2) which drive the expression of cre-recombinase. All mice were housed in pathogen-free conditions in the same designated room of the animal facility at H. Lee Moffitt cancer center and The

George Washington University. All animal studies were performed according to the guideline of approved protocols by the Institutional Animal Care and Use Committee

(IACUC) at the University of South Florida and The George Washington University.

EL-4 murine thymoma was purchased from ATCC and cultured and maintained in Dulbecco’s modified Eagle’s medium (DMEM, Corning) supplemented with 4 mM L-

72 glutamine, 4500 mg/L glucose, 1mM sodium pyruvate, 1500 mg/L sodium bicarbonate,

10% horse serum (ATCC), 100 U/mL penicillin and 100 µg/mL streptomycin. Cell line

was grown under humidified conditions at 5% CO2 and 37ºC. Cells were frozen at early passages (less than 6) in 95% culture medium + 5% DMSO and were used within 30 days of recovering.

3.4.2 Genotyping: To identify the genotypes of HDAC11KO, HDAC11 flox/flox and Lyz-CreHDAC11 conditional KO mice, total genome DNA was isolated and a polymerase chain reaction (PCR) experiment was performed with specific forward and reverse primers.

Briefly, approximately 0.5 cm of tail snips was obtained from each mouse and

then digested overnight in a lysis buffer containing 10 mM Tris-HCl (PH 8.0), 50 mM

NaCl, 25 mM EDTA, 0.5% SDS and 10 mg/mL proteinase K at 55 ºC. 6M NaCl was added to each sample followed by 15 seconds of vortexing then 30 minutes of centrifugation at 13,000 g at room temperature. The supernatant was transferred to a new conical tube containing 100% ethanol and DNA precipitated by inverting the tube.

After spinning down at 13,000 g for 10 minutes, DNA pellets were washed once with

75% ethanol and then resuspended in Tris-EDTA buffer. DNA concentration and quality was determined.

Primer sequences used for each strain are as follows: HDAC11KO Forward

(TGCTGCCTGTGAGCCACTGC), Reverse (CCTTGGAATAGCATCTCAGG). HDAC11-

flox Forward (TGCTGCCTGTGAGCCACTGC), Reverse

(AGAATGGCTGTCTCCCTAAG). Lyz-Cre set 1) WT (TTACAGTCGGCCAGGCTGAC),

73 Common (CTTGGGCTGCCAGAATTTCTC). LyZ-Cre set 2) Mutant

(CCCAGAAATGCCAGATTACG), Common (CTTGGGCTGCCAGAATTTCTC). For

each 25 µL PCR reaction, we used 0.5 µM of forward or reverse primer, 2.6 mM MgCl2,

0.26 mM dNTPs, 0.75U taq polymerase and 50 ng of DNA was used and product size was resolved on a 1.5% acrylamide gel containing SYBR safe DNA Gel Stain

(Invitrogen).

3.4.3 In Vivo tumor model: For in vivo tumor studies, 0.25×106 EL-4 cells were

injected subcutaneously into the right flank of female mice that were 8 weeks old.

Tumor volume was measured starting around Day 8 after injection or at the time when

tumors were palpable.

3.4.4 Splenic and tumor infiltrated MDSCs isolation: Mouse spleens or

tumors were harvested under sterile conditions on Day 21 after subcutaneous injection.

Total splenocytes were obtained by mashing mouse spleens through a 70 µm sterile cell strainer (Fisher Scientific). Mouse tumor tissue was cut into small pieces followed by incubation with tumor dissociation solution (RPMI-1640 supplemented with 10%

FBS, 0.25 mg/mL Collagenase D and 60 U/mL DNase I) for 30 minutes at 37 ºC. The disassociated tumor cells were collected by straining through a 70 µm sterile cell strainer. Red blood cells from spleen and tumor cells were lysed by incubating with ACK lysis buffer (Gibco) for 3 minutes at room temperature. Cells were washed twice with

PBS subsequently. MDSCs were further purified by magnetic bead isolation. Briefly, cells obtained from spleens or tumors were incubated with biotinylated anti-Gr-1

antibody (Invitrogen) followed by streptavidin microbeads (Miltenyi) for 15 minutes each

74 on ice. Gr-1+ MDSCs were isolated on LD columns (Miltenyi) according to the

manufacturer’s instruction.

3.4.5 BM-derived MDSCs generation: Mouse bone marrow aspirate was

removed from the tibias and femurs by flushing with ice-cold PBS and RBCs were

removed using ACK lysis buffer. To obtain bone marrow derived MDSCs, 5×106 bone

marrow cells were plated into 100 mm diameter tissue culture dishes in 10 mL RPMI-

1640 medium supplemented with 10% FBS, 100 U/mL penicillin, 100 µg/mL

streptomycin, 10 mM HEPES (Corning), 50 uM 2-mercaptoethanol (gibco), 40 ng/mL

mouse recombinant GM-CSF (Peprotech Inc) and 40 ng/mL mouse recombinant G-CSF

(Peprotech Inc). Cells were maintained in a humidified atmosphere with 5% CO2 at 37

ºC for 4 days. Fresh medium was replaced from cultured cells at Day 3 [31]. Gr-1+

MDSCs were purified by using magnetic bead isolation for each day as previously

described.

3.4.6 Arginase activity assay: To determine arginase activity, an arginase activity assay kit was used according to the manufacturer’s instructions (Sigma-Aldrich).

Briefly, 1×106 freshly isolated MDSCs were lysed in 100 µL lysis buffer containing 10

mM Tris-HCl (pH 7.4), 0.4% (w/v) Triton X-100 (Fisher), 1 µM pepstatin (Sigma-Aldrich)

A and 1 µM leupeptin (Sigma-Aldrich) for 10 minutes on ice. Cell lysate was collected by

centrifugation at 13,000 g for 10 minutes at 4 ºC. 40 µL of cell lysate was added to each

of two wells of a 96 well plate, one each serving for reaction well and blank well. 50 µL

of 1mM urea was added to the late serving for standard. 10 µL of 5× substrate buffer (8

µL arginase buffer + 2 µL Mn solution) was then added to each sample reaction well

and the plate was incubated for 120 minutes at 37 ºC. To stop the arginase reaction,

75 200 µL of urea reagent (100 µL reagent A + 100 µL reagent B) was added to each well

and 10 µL of 5× substrate buffer was added to sample blank wells. The plate was

incubated for 45 minutes at room temperature and urea concentration was defined by

measuring the absorbance at 430 nM using a microplate reader (Bio-Rad). One unit of

arginase activity is defined as the amount of enzyme that catalyzes the formation of 1

µmole of L-arginine to ornithine and urea per minute.

3.4.7 NO production assay: To detect nitrite production, 0.5 × 106 freshly

isolated MDSCs were cultured in a 96 well plate with 150 µL RPMI-1640 medium

supplemented with 10% FBS and 10 ng/mL mouse recombinant GM-CSF for 24 hours

in the presence or absence of 100 ng/mL mouse recombinant IFN-γ. 100 µL of culture supernatant were mixed with equal volume of Greiss reagent containing 1% (100 mg) sulfanilamide (Sigma-Aldrich), 0.1% (10 mg) N-(1-naphthyl)ethylenediamine (Sigma-

Aldrich) and 5% (0.5 mL) H3P04 (Fisher). After incubation for 10 minutes at room

temperature, the absorbance was measured at 550 nm using a microplate reader.

Nitrite concentration was measured by comparing the absorbance values for the

samples to a standard curve generated by 8 serial dilutions of 0.25 mM sodium nitrite

(Sigma-Aldrich).

3.4.8 ROS production: Oxidation-sensitive dye DCFDA (Invitrogen) was used to measure reactive oxygen species production by MDSCs. 2 to 3 ×106 freshly isolated

BM-derived MDSCs, splenocytes or MDSC-infiltrated tumor tissue digestions collected

from tumor bearing mice were labeled with flow antibodies against surface markers as

following: CD11b (M1/70, BD Biosciences), Ly6G (1A8, BD Biosciences) and Ly6C (AL-

21, BD Biosciences) for 20 minutes at 4ºC. Cells were then incubated in 500 µL serum

76 free RPMI-1640 medium with 3 µM DCFDA for 30 minutes in dark at 37ºC, in the

presence or absence of 300 nM PMA (Sigma-Aldrich). The cells were analyzed using

flow cytometry.

3.4.9 Quantitative Real-time PCR (qRT-PCR): Total messenger RNA was

extracted using Trizol (Invitrogen) according to manufacturer’s instruction. cDNA was

synthesized from 1 µg of mRNA for each sample by using iScript cDNA synthesis kit

(Bio-Rad). To detect gene expression, qRT-PCR was performed using a SYBR Green

two-step system. Primers targeting specific genes were designed or purchased as follows: Murine C/EBPα Forward (CCCACTCAGCTTACAACAGG), Murine C/EBPα

Reverse (GCTGGCGACATACAGTACAC). Murine C/EBPβ Forward

(ACACGTGTAACTGTCAGCCG), Murine C/EBPβ Reverse

(GCTCGAAACGGAAAAGGTTC). Murine HDAC11 Forward

(AGAGAAGCTGCTGTCCGATG), Murine HDAC11 Reverse

(AGGACCACTTCAGCTCGTTG). Murine 18s rRNA (QT 02448075, QuantiTech Primer

Assay, Qiagen).

3.4.10 Flow cytometry analysis and cell sorting: For flow cytometric analysis,

cells were stained with fluorochrome-labeled monoclonal antibodies (mAbs; anti-CD11b,

-Ly6G, -Ly6G, BD Biosciences) for 20 minutes at 4ºC and washed for 2 times with

FACS buffer. The vitality dye 4', 6-diamidino-2-phenylindole (DAPI, 50 ng/mL, Sigma)

was added to cells before analysis. Data was acquired on FACSCelesta flow cytometer

(BD Biosciences), at least 10,000 events, of the smallest population of interest,

subsequently analyzed using FlowJo software 10.2.

77 3.4.11 Western blotting analysis: Approximately 5 to 10 ×106 MDSCs were lysed in RIPA buffer containing 50 mM Tris-HCl (pH. 8.0), 150 mM NaCl, 0.5% Sodium

Deoxycholate, 0.1% SDS, 1% Triton X-100 with freshly added protease inhibitor

(Sigma-Aldrich), phosphatase inhibitor (Santa Cruz Biotechnology). The cell lysates were left on ice for 30 minutes followed by 2 cycles of sonication (8 min 30s on/30s off for each cycle. The lysates were then mixed with 2× gel loading buffer and boiled for 10 minutes. Samples were then resolved on 4 to 10% SDS-PAGE gels (Bio-Rad) and transferred to nitrocellulose membranes. Membranes were blocked with buffer containing 5% milk and 0.05% Tween-20 (Sigma-Aldrich) and probed with appropriate primary antibodies overnight at 4ºC. Membranes were then washed three times with wash buffer and incubated with secondary antibody for one hour at room temperature.

Bands were visualized by chemiluminescence detection on Azure Biosystem. To confirm equal loading, anti-GAPDH (Sigma Aldrich) was used for each experiment. Anti-

C/EBPβ, anti-Arginase and anti-iNOS antibodies were purchased from abcam.

3.4.12 Adoptive Cell Therapy: In this model, female C57BL/6 and HDAC11KO

mice (7 to 8 weeks old) were injected with 0.25×106 EL4 cells subcutaneously into

shaved right flanks on Day 0. A mixture of MDSCs (5×106/mouse) and CD3+ T cells

(5×106/mouse) from was intravenously injected to the animals on Day 0 and Day 7 upon

EL4 tumor inoculation, as described previously [186]. Briefly, on day of adoptive

transfer, spleens were harvested from C57BL/6 and HDAC11KO EL4 bearing mice. WT

or HDAC11KO MDSCs were labeled with biotinylated anti-Gr-1 antibody and streptavidin magnetic beads and positively selected on a LD column. Meanwhile, lymph

78 nodes and spleens from naïve C57BL/6 and HDAC11KO mice were harvested and

single cell suspensions was obtained after being lysed for red blood cells. CD3+T cells

were purified using a negative selection kit (EasySep, STEMCELL technology). Both

MDSCs and T cells were washed twice with ice cold PBS and re-suspended in HBSS at

a concentration of 5×106 cells/150 µL for I.V. injection.

3.4.13 Chromatin Immunoprecipitation (ChIP): Mouse MDSCs (20×106) were

cross-linked with 1% formaldehyde (Sigma-Aldrich) for 10 minutes at room temperature.

The cross-linking was then stopped with 0.125 M glycine for 10 minutes at room

temperature. The cells were washed twice with PBS and proceed with sample

preparation. The cell pellet was lysed by 1 mL of lyse buffer (50 mM HEPES pH 7.8, 3

mM MgCl2, 20 mM KCl, 0.25% Triton X-100, 0.5% NP-40) on ice for 10 minutes. The cell lysate was then transferred to a “Dounce Homogenizer” and 20 strokes were applied using the loose pestle A. The cell pellet was washed once with 1 mL wash buffer (10 mM Tris-HCl pH 8.0, 1 mM EDTA, 200 mM NaCl) and resuspended in 300 µL sonication buffer (50 mM HEPES pH 7.8, 140 mM NaCl, 1 mM EDTA, 0.5% SDS, 1%

Triton X-100). 8 sonication cycles (8 minutes of 30s on/off) were applied to the samples

followed by centrifugation for 10 minutes at 4ºC. The lysate was diluted at 1:10 with

dilution buffer I (50 mM HEPES pH 7.8, 140 mM NaCl, 1mM EDTA, 1% Triton X-100),

pre-cleared using Protein A Agarose beads (Millipore), and incubated with rabbit anti-

HDAC-11 antibody cocktail (BioVision and Sigma-Aldrich) on a rotator at 4°C overnight.

Biotin-conjugated normal rabbit IgG-B (Santa Cruz Biotechnology) was added at the

same amount of rabbit anti-HDAC-11 antibody cocktail in parallel of each sample and

incubated on a rotator at 4°C overnight. 50 µL protein A Agarose beads was then

79 applied to each sample and incubated at 4°C for 4 hours. The beads were then washed

twice each with dilution buffer I, dilution buffer II (50 mM HEPES pH 7.8, 500 mM NaCl,

1 mM EDTA, 1% Triton X-100), LiCL buffer (20 mM Tris-HCl pH 8.0, 250 mM LiCl, 1

mM EDTA, 0.5% Triton X-100), and TE buffer (10 mM Tris-HCl pH 8.0, 1 mM EDTA).

Afterwards, beads were eluted in 100 µl elution buffer (50 mM Tris-HCl pH 8.0, 1 mM

EDTA, 1% SDS) and incubated at 65°C for 15 minutes and the immunocomplexes were collected in the soluble fraction. NaCl was added to a final concentration of 200 mM and the crosslinking was reverted by incubating at 65°C for overnight. RNAse A (Qiagen) was added to 20 µg/mL to each sample and incubated at 37°C for 30 minutes.

Proteinase K (Thermo Scientific) was then added at 100 µg/mL and incubated at 42°C for 2 hours. DNA was purified by using QIAquick PCR Purification Kit (Qiagen) and purified DNA was eluted in 100 µL TE buffer for analysis by qRT-PCR. 4 µL of DNA was used for each qRT-PCR reaction. The following PCR primers were used: C/EBPβ

Forward (ATGCACACAGGCAAGTTTCA), C/EBPβ Reverse

(TCTCTCAGGCCTCAGTTTCC).

3.4.14 Statistics: Statistical analyses were performed using student’s t test. Data expressed as the mean ± SD. Probability values of p ≤ 0.05 were considered significant.

3.5 Results

3.5.1 C/EBPβ is necessary for bone marrow-derived and tumor-induced

MDSCs.

80 Previously in our group, we have demonstrated that when compared with naive

myeloid cells, HDAC11 expression was significantly reduced in the myeloid-derived

suppressor cells in response to tumor challenge. Without HDAC11, MDSCs are

functionally more suppressive by implicating antigen-specific CD8+ T cell

unresponsiveness in vitro [155]. However, the mechanism underlying this functional

characteristic remains uninvestigated. C/EBP transcription factor family is largely

involved during myelopoiesis. C/EBPα is a "master regulator" of granulopoiesis at steady-state, whereas C/EBPβ is required for emergency granulopoiesis in response to cytokines and infection [187]. It wasn't until 2010, Marigo and colleagues had first

uncovered the crucial role of C/EBPβ in regulating the immunosuppressive activity of

MDSCs in tumor bearing host. Complete deletion of C/EBPβ resulted in full abrogation

of MDSCs on antigen specific CD8+ T cells, possibly through reduction of expression

and reduced enzymatic activity of arginase 1 and nitric oxide synthase 2, two enzymes

that have previously been described as major contributors to the inhibitory ability of

MDSCs [31, 182].

To determine whether or not C/EBPβ is involved with the elevated suppressive

activity of HDAC11KO MDSCs, we assessed the gene expression profile of C/EBPβ in

WT or HDAC11KO MDSCs. We isolated CD11b+Ly6G+Ly6Clow and CD11b+Ly6G-

Ly6Chigh cells from spleens of naive and tumor-bearing C57BL/6 WT animals (Figure

3.1A). C/EBPβ mRNA is barely detectable in immature myeloid cells from naive mice.

Interestingly, both CD11b+Ly6G+Ly6Clow granulocytic-MDSCs and CD11b+Ly6G-

Ly6Chigh monocytic-MDSCs from tumor bearers showed an elevated C/EBPβ mRNA

expression compared with IMCs sharing the same surface phenotype from naive mice,

81

Figure 3.1 C/EBPβ expression is up-regulated in MDSCs with HDAC11 deletion. (A) In Naïve and EL4 tumor bearing C57BL/5 mice, splenic monocytes (Ly6G+Ly6Chigh cells in naïve mice) and monocytic MDSCs (Ly6G+Ly6Chigh cells in tumor bearing mice), as well as splenic neutrophils (Ly6G+Ly6Clow cells in naïve mice) and granulocytic MDSCs (Ly6G+Ly6Clow cells in tumor bearing mice) were flow sorted. C/EBPβ expression was further assessed by qRT-PCR. (B) Ly6G+Ly6Clow Monocytes and Ly6G-Ly6Chigh neutrophils were flow sorted from the bone marrow of C57BL/6 mice and HDACKO mice. The expression of C/EBPβ profile were analyzed using qRT-PCR . (C) Gr-1+ bone marrow derived-myeloid cells (day o) and –MDSCs (day 4) were isolated using biotinylated anti-Gr-1 antibody and magnetic beads. C/EBPβ mRNA expression was determined by qRT-PCR. Error bars = S.E.M. Data presented for each graph is a representative figure from 2 individual experiments, 3 animals were used for each experiment. Asterisk indicates statistical significance: *P<0.05, **P<0.01, ***P<0.001.

82 suggesting that up-regulation of C/EBPβ is required during the transition of immature

myeloid cells to MDSCs. We then flow-sorted CD11b+Ly6G+Ly6Clow and CD11b+Ly6G-

Ly6Chigh cells from WT and HDAC11KO bone marrow (Figure 3.1B). To our surprise, we

have found out that at steady state, C/EBPβ level was significantly higher for both

Ly6G+ and Ly6C+ IMCs lacking HDAC11. To further confirm this, we isolated BM-

derived Gr-1+ MDSCs and found up-regulated mRNA expression of C/EBPβ in

HDAC11KO MDSCs. Given Marigo's data that C/EBPβ-deleted myeloid cells failed to

generate functional MDSCs in vivo, we hypothesize that HDAC11KO myeloid cells are

more suitable for MDSC generation with its abnormally high level of C/EBPβ.

3.5.2 Elevated level of C/EBPβ contributes to the up-regulation of enzymes

involved with MDSC suppression in HDAC11KO mice.

We next evaluated whether up-regulation of C/EBPβ in HDAC11KO MDSCs

might control suppressive immunoregulatory pathways. We isolated bone marrow cells

from WT and HDAC11KO mice followed by a treatment with 40 ng/mL of GM-CSF and

G-CSF for up to four days in vitro. Gr-1+ cells were then purified after 72 and 96 hours

and immunoblottings of targets of interest were performed (Figure 3.2A). We found that

consistent with our mRNA result, C/EBPβ protein was not detectable in Gr-1+ bone

marrow cells at day 0 of harvest. On both day 3 and day 4, an increased C/EBPβ

protein level was observed in HDAC11KO MDSCs along with an over-expression of

arginase 1 (Arg1) and nitric oxide synthase 2 (Nos2). The data was further confirmed in

splenic-MDSCs and tumor infiltrated-MDSCs isolated from WT and HDAC11 KO tumor-

83

Figure 3.2 C/EBPβ is involved with the suppressive activity of MDSCs lacking HDAC11. (A) Immunoblots of C/EBPβ, Arginase 1 and NOS2 in Gr-1+ MDSCs isolated from WT and HDAC11KO mice fresh bone marrow (indicated as day 0) and BM cells treated with GM-CSF and G-CSF for 72 and 96 hours (day 3 and day 4). (B) Immunoblots of C/EBPβ in Gr-1+ MDSCs isolated from spleens and tumors of WT or HDAC11KO mice bearing EL4 tumor at Day 21. LAP*, LAP and LIP indicate three isoforms of C/EBPβ protein.

84 bearing mice. As shown in Figure 3.2B, C/EBPβ, in the absence of HDAC11, Arg1 and

Nos2 protein level were increased in both splenic- and tumor infiltrated- MDSCs.

Interestingly, C/EBPβ and Nos2 displayed a stronger expression profile in MDSCs isolated from tumor site, where as Arg1 level seems to be similar in between these two sources. Earlier studies had pointed out the transcriptional regulation by C/EBPβ of

Arg1 and Nos2 genes [188-190]. In Marigo's recent work, loss of C/EBPβ results in a reduction to near complete depletion of both Arg1 and Nos2 protein in MDSCs [31].

With these observations, we believe that the loss of HDAC11 is associated with the up- regulation of Arg1 and Nos2 through transcription factor C/EBPβ.

3.5.3 HDAC11 deletion promotes MDSCs suppressive activity through

increased arginase activity and NO production.

Previous studies had demonstrated that arginase activity, NO and ROS production were the main effectors of MDSC's suppressive function. Up-regulation of Arginase 1 activity results in rapid uptake of L-arginine from the tumor microenvironment which contributes to the loss of the CD3ζ chain and prevents T cell proliferation [48, 162, 182]. Increased

NO synthase expression can facilitate MDSCs to produce more NO and suppress T cell function through down-regulated downstream pathways of IL-2 receptor [162, 191].

Hyperproduction of reactive oxygen species (ROS) such as hydrogen peroxide and peroxynitrite can induce T cell apoptosis [42]. In our earlier finding, MDSCs devoid of

HDAC11 can prevent IFN-γ production in antigen- specific CD8+ OTI T cells in responding to OVA peptide. HDAC11 deficient mice displayed an enhanced tumor

85 growth pattern which indicates a more suppressive microenvironment in those tumor

bearing animals when compared with WT.

To further compare the suppressive functional activity of WT and HDAC11KO

MDSCs, we performed arginase activity and NO production assays (Figure 3.3 and

Figure 3.4). Bone marrow cells were collected from WT and HDAC11KO mice followed by GM-CSF and G-CSF treatment for four days. Gr-1+ cells were isolated at each day

Figure 3.3 Enzymes involved in the suppressive pathways employed by BM-derived MDSCs with HDAC11 deletion. (A) Gr-1+ MDSCs were isolated from bone marrow and treated with GM-CSF and G-CSF for 24, 48, 72 and 96 hours (day 1, 2, 3 and 4). The enzymatic activity of Arginase was evaluated from cell lysate as previously described. (B) Bone marrow-derived Gr-1+ MDSCs from WT or HDAC11KO mice were also plated in complete RPMI-1640 medium containing GM-CSF, with or without IFN-γ for 24 hours. Cell supernatants were collected and assayed for NO production to determine Nos2 activity. Error bar = S.E.M. Asterisk indicates statistical significance: *P<0.05, **P<0.01, ***P<0.001.

86 and arginase activity evaluated from cell lysate. Accordingly, arginase activity was

significantly increased in HDAC11KO MDSCs on both day 3 and 4 (Figure 3.3A). After

24 hours of IFN-γ stimulation, NO production was measured from culture medium.

HDAC11KO MDSCs facilitated significant increase of NO than WT on day 3 and day 4

(Figure 3.3B).

Next we performed similar experiments in splenic- and tumor infiltrated-MDSCs

(Figure 3.4). Interestingly, splenic-MDSCs isolated from WT or HDAC11KO tumor bearing mice showed a similar (arginase activity) or moderately increased (NO production, not significant) activity from both enzymes (Figure 3.4A and 3.4B). However, upon entry into the tumor site, HDAC11KO MDSCs displayed a dramatic increase in arginase activity and NO production, indicating abnormal activity from both enzymes in cells depleted of HDAC11 (Figure 3.4A and 3.4B). Cytokines such as GM-CSF, G-CSF and IL-6 produced by tumor are some of the main factors that promote the suppressive function and differentiation of MDSCs [43, 192]. Given our previous observation that

C/EBPβ protein levels are significantly higher in tumor infiltrated-MDSCs than in splenic-

MDSCs, we believe that in response to cytokines such as GM-CSF and G-CSF, up- regulation of C/EBPβ in HDAC11KO MDSCs facilitates their ultra suppressiveness in the tumor microenvironment.

87

Figure 3.4 Enzymes involved in the suppressive pathways employed by HDAC11KO MDSCs in vivo. (A) Gr-1+ MDSCs were isolated from spleens and tumors of C57BL/6 WT or HDAC11KO mice bearing EL4 tumors at day 21. The enzymatic activity of Arginase was evaluated from cell lysate as previously described. (B) Gr-1+ MDSCs from WT or HDAC11KO tumor bearers were also plated in complete RPMI-1640 medium containing GM-CSF, with or without IFN-γ for 24 hours. Cell supernatants were collected and assayed for NO production to determine Nos2 activity. Tumors were collected on day 21 after EL4 inoculation. Cells were stimulated with or without PMA in RPMI medium for 30 minutes then labeled with anti-CD11b, anti-Ly6G and anti-Ly6C antibodies. ROS was measured in G-MDSCs (C) and M-MDSCs (D) population by labeling cells with DCFDA. Mean florescence intensity (MFI) is shown in each indicated

88 figure. Error bar = S.E.M. Asterisk indicates statistical significance: *P<0.05, **P<0.01, ***P<0.001.

3.5.4 HDAC11 negatively regulates C/EBPβ expression by interacting with

the promoter region of this transcription factor.

Given our presented data so far, we hypothesize that HDAC11 could be a

negative regulator of C/EBPβ. The higher mRNA expression of C/EBPβ in HDAC11KO

immature myeloid cells may indicate that those IMCs are ready to differentiate into

functional suppressive MDSCs when recruited into the tumor microenvironment. To

further elaborate the mechanism of how HDAC11 regulates C/EBPβ, we performed

chromatin-immunoprecipitation analysis. Gr-1+ MDSCs isolated from C57BL/6 WT and

HDAC11KO tumor-bearing mice were cultured in medium for 3 hours with or without

LPS. ChIP data suggest that HDAC11 was present on the promoter region of C/EBPβ in

WT MDSCs (Figure 3.5A), with an enrichment ratio at least 2.5 fold higher than

HDAC11KO MDSCs (Figure 3.5B) which serves as a negative control. These data

suggest that HDAC11 could alter the expression of C/EBPβ through chromatin

remodeling or simply function as a transcription factor. Unlike other HDACs, there has

been no literature reporting on HDAC11’s ability to bind to DNA directly or, coupled with

other proteins, to directly facilitate transcription initiation or regulation. At this point we

could not fully answer the question of how HDAC11 negatively regulates C/EBPβ. A

more in-depth study is required to fully understand the mechanism.

89

Figure 3.5. HDAC 11 regulates C/EBPβ expression in MDSCs. MDSCs were purified from C57BL/6 mice and HDACKO mice for Chromatin-immunoprecipitation. ChIP studies showed the presence of HDAC11 protein to the promoter region of C/EBPβ in C57BL/6 mice MDSCs (A) but not MDSCs from HDAC11 KO mice (B). Error bars = S.E.M. Asterisk indicates statistical significance: *P<0.05, **P<0.01, ***P<0.001.

3.5.5 Mice with HDAC11 deletion in myeloid compartment displayed

enhanced tumor progression compared to total knockout mice.

Our previous work demonstrated an enhanced tumor progression pattern in

HDAC11KO tumor bearers. Woods and Woan in our lab had found out that T cells possessed a hyper reactive phenotype and enhanced anti-tumor efficacy in vivo [82]. In

their study, FCmuMCL tumor bearing mice established a significant delay in tumor

growth and reduced tumor volume when adoptively transferred with HDAC11KO T cells

[82]. We thought the HDAC11 total knockout mouse might not serve as the best model

to study MDSCs function in vivo. Taking that into consideration, we decided to establish

a mouse model with a linage-specific HDAC11 knockout in the myeloid compartment

only. In Figure 3.6, our in vivo experiment show that upon EL4 tumor challenge, mice

90 with HDAC11 myeloid-lineage conditional knockout displayed enhanced tumor growth

when compared with HDAC11 total knockout and WT mice, possibly resulting from the

absence of HDAC11KO T cells in the tumor microenvironment. However, tumor grew larger in HDAC11 total knockout mice compared with WT mice, indicating that although the hyperactive HDAC11KO T cells could function against tumor progression, but when encountered with HDAC11KO MDSCs, at least in this model, their anti-tumor capability were limited.

Figure 3.6 Mice lacking HDAC11 in the myeloid compartment demonstrate a more enhanced tumor growth pattern than HDAC11 total knock out. 0.25×106 EL4 cells were subcutaneously injected to C57BL/6 mice, HDAC11KO mice and HDAC11KO Cre-LyZ mice (n=5 per group) for up to 19 days. Tumor volume was measured for every 3 days after the initiation of tumors. The tumor growth pattern was analyzed to compare HDAC11 total KO mice with mice lacking HDAC11 in the myeloid compartment (HDAC11 KO Cre-LyZ), HDAC11 KO mice with C57BL/6 WT mice and HDAC11KO

91 Cre-LyZ with C56BL/6 mice. (Error bar = S.E.M). Asterisk indicates statistical significance between WT and HDAC11 KO, WT and HDAC11 KO Cre-LyZ, HDAC11KO and HDAC11KO Cre-LyZ tumor bearing mice: *P<0.05, **P<0.01, ***P<0.001.

3.5.6 HDAC11 plays a contradictory role in regulating MDSCs and T cells in

the tumor microenvironment.

To further investigate the contradictory role of HDAC11 plays in MDSCs and T

cells, we performed an in vivo experiment where we adoptively transferred a single cell

type or a mixture of MDSCs and T cells (from WT or HDAC11KO) to C57BL/6 EL4

tumor bearers. Briefly, on day 0, 9 groups of C57BL/6 mice were inoculated with EL4

tumor cells subcutaneously. Two adoptive transfers of MDSCs and/or T cells were given

to indicated mice on day 0 and day 7. In figure 3.7A, we found out that adoptive

transfer of HDAC11KOT cells into EL4 tumor bearing mice can significantly reduce

tumor progression than mice receiving WT T cells. Notably, tumor volume of mice

receiving HDAC11KO MDSCs alone is significantly larger than mice that received WT

MDSCs, which is expected given the enhanced suppressive phenotype of those cells

(Figure 3.7B). Additional transfer of HDAC11KO T cells significantly reduced both

MDSC-promoted tumor growth in mice receiving WT MDSCs or HDAC11KO MDSCs

(Figure 3.7C, 3.7D). However, the anti-tumor effect by HDAC11KO T cells was affected

when they encounter HDAC11KO MDSCs in the tumor microenvironment (Figure 3.7E,

3.7F).

92

Figure 3.7 The antitumor efficacy by adoptive transfer of HDAC11KO T cells can be diminished by MDSCs lacking HDAC11. 0.25 × 106 EL4 cells were subcutaneously injected to 9 groups of C57BL/6 mice on day 0 (n=5 per group). On day 0 and day 7, mice were given WT MDSCs, H11KO MDSCs, WT T cells, H11KO T cells alone or a mixture of WT or 11KO MDSCs with naive WT or 11KO T cells intravenously. For each mouse receiving adoptive transfer, 5 × 106 MDSCs or T cells were used. One group of mice receiving only EL4 tumor cells served as No i.v control. Tumor volume was measured for every 2 days after the initiation of tumors. The tumor growth pattern was analyzed in comparison with (A) C57BL/6 tumor-bearing mice with adoptive transfer of

93 C57BL/6 WT T cells alone, HDAC11KO T cell alone and No i.v control, (B) C57BL/6 tumor-bearing mice with adoptive transfer of WT MDSCs alone, HDAC11KO MDSCs alone and No i.v control, (C) C57BL/6 tumor-bearing mice with adoptive transfer of WT MDSCs alone, WT MDSCs + WT T cells, WT MDSCs + HDAC11 KO T cells and No i.v control, (D) C57BL/6 tumor-bearing mice with adoptive transfer of HDAC11KO MDSCs alone, HDAC11KO MDSCs + WT T cells, HDAC11KO MDSCs + HDAC11KO T cells and No i.v control. (E) C57BL/6 tumor-bearing mice with adoptive transfer of WT MDSCs + WT T cells, HDAC11KO MDSCs + WT T cells, WT T cells alone. (F) C57BL/6 tumor-bearing mice with adoptive transfer of WT MDSCs + HDAC11KO T cells, HDAC11KO MDSCs + HDAC11KO T cells, HDAC11KO T cells alone. (Error bar = S.E.M). Asterisk indicates statistical significance: *P<0.05, **P<0.01, ***P<0.001.

3.6 Discussion

Myeloid-derived suppressor cells represent a heterogeneous population of

immature myeloid cells that are recruited to the tumor microenvironment where they

differentiate into immunosuppressive cells. As one of the main mechanisms that

facilitate the immune evasion of cancer, MDSCs have garnered a great deal of interest

from scientific investigators and were believed to be a potent target for therapeutic

approaches against cancer. Here we showed for the first time that among all the

HDACs known, through negative regulation of C/EBPβ, HDAC11, the newest member

of this family of enzymes, promotes the suppressive function of MDSCs in mouse

model.

C/EBPβ had been recently demonstrated to control emergency granulopoiesis

induced by cytokines such as GM-CSF or infection [187]. It is the discovery of C/EBPβ

deletion could mostly eliminate MDSC suppressive activity until investigators first

associate this molecule with MDSC development and biological function [31]. Previously

we have found out that in the absence of HDAC11, MDSCs display an enhanced

suppressive phenotype against antigen-specific CD8+ T cell function and promote tumor

94 progression [155]. However, little is known regarding the C/EBPβ expression profile in

MDSCs devoid of HDAC11. In our study, we observed an increased C/EBPβ level in

HDAC11KO BM-derived MDSCs compared with WT. C/EBPβ is generally not

detectable in myeloid cells at steady state. To our surprise however, we detected a

markedly higher expression of C/EBP-β mRNA in both CD11b+Ly6G+Ly6Clow and

CD11b+Ly6G-Ly6Chigh compartments at steady state in HDAC11KO vs WT control,

indicating a possibility of HDAC11KO myeloid cells being primed and ready to

differentiate into functional MDSCs (Figure 3.1). Additionally, in the absence of

HDAC11, we have detected an increased protein level of C/EBPβ in in vitro cultured

BM-derived MDSCs, as well as splenic and tumor-infiltrated MDSCs from our in vivo

tumor model when compared with WT (Figure 3.2). Moreover, ChIP analysis results

suggest that HDAC11 was recruited to the promoter region of C/EBPβ, indicating a

direct or indirect transcriptional machinery involved with HDAC11 (Figure 3.5). Along with that, two enzymes known to be associated with C/EBPβ, arginase 1 and nitric oxide

synthase 2 showed increased protein level in HDAC11KO MDSCs compare with WT

(Figure 3.2). In MDSCs, arginase activity, NO and ROS production are the main

effectors to suppress T cell function. We next assessed enzymatic activity analysis for

those factors. Our data suggest that in the absence of HDAC11, in vitro GM-CSF and

G-CSF induced MDSCs displayed a higher arginase activity and NO production (Figure

3.3). Similarly, tumor-infiltrated HDAC11KO MDSCs had a dramatic increase of both

Arg1 activity and NO, but not ROS production (Figure 3.4). However, both arginase activity and NO level were at a similar level in the splenic-MDSCs from tumor bearing mice. Both Arg1 and iNOS use L-arginine as a substrate and the uptake of L-arginine

95 from the tumor microenvironment can suppress T cell function through multiple

pathways [48, 193, 194]. Taken together, our data suggest that through arginine

metabolism regulated by C/EBPβ-induced enzyme Arg1 and Nos2, HDAC11KO MDSCs

function more suppressive in the tumor microenvironment.

In addition to having increased suppressive phenotypes in HDAC11KO MDSCs,

studies have shown that T cells lacking HDAC11 had enhanced effector function

against tumor [82]. Our observation in HDAC11 myeloid-lineage conditional knockout provides evidence to support this finding. In solid tumor, the microenvironment consist of a heterogeneous population of tumor cells, stromal cells, fibroblasts and a mixture of different immune cells such as T cells, MDSCs, tumor associated macrophages and regulatory T cells. Deletion of HDAC11 in the knockout mouse will inevitably affect all cell types and will bring an uncertainty in how to interoperate our data. Using the

HDAC11 Cre-LyZ conditional knockout model allow us to investigate the suppressive function of MDSCs without interference from HDAC11KO T cells. When challenged with tumor, the HDAC11 Cre-LyZ mice indeed showed a dramatic increase in tumor progression compared with total knockout mice (Figure 3.6). This finding provides evidence to allow us to further investigate the contradictory role HDAC11 played in the tumor microenvironment.

In our in vivo study, tumor progression in C57BL/6 tumor bearing mice receiving

HDAC11KO T cells was significantly reduced than mice receiving WT T cells, whereas adoptive transfer of HDAC11KO MDSCs alone could promote tumor growth (Figure

3.7A, 3.7B). Additional transfer of HDAC11KO T cells could significantly reduce both

MDSC-promoted tumor growth in mice receiving WT MDSCs or HDAC11KO MDSCs

96 (Figure 3.7C, 3.7D). However, the anti-tumor effect by HDAC11KO T cells was affected

and reduced when co-existing with HDAC11KO MDSCs. When encountered with both

HDAC11KO and WT T cells, WT MDSCs appeared to have a milder suppression

(Figure 3.7E, 3.7F). Based on our data, we hypothesize that to gain a full anti-tumor effect from T cells by HDAC11 inhibition, it is necessary to eliminate MDSC function or infiltration.

A growing body of evidence has demonstrated that targeting HDAC11 could be a potent therapeutics against in multiple cancer cell lines or across different cancer types

[84, 87, 195]. There are a few HDAC11 inhibitors commercially available and hopefully more will be generated with a higher specificity. Inhibition of HDAC11 activity either as stand-alone or in combination with other therapies will inevitably target the host immune system and tumor cells simultaneously. With its potent ability to target cancer cells directly, the additional effect on T cell function could boost the anti-tumor capability of the drugs. However, this effect could be potentially jeopardized by the benefit of

HDAC11 inhibition to MDSCs. In addition to HDAC11 inhibition blockade of MDSC infiltration or elimination of its function could potentially provide a better outcome as a new therapeutic approach. Growing interest has focused on MDSC inhibition in cancer patients. Targeting GM-CSF signaling or CSF-R blockade could potentially benefit chemotherapy and reverse resistance to immunotherapy in vitro [196, 197]. Inhibiting

MDSC NO production through Nrf2 degradation by RTA 408, which is currently being evaluated in clinical trials, benefits the outcome of treatment with its anti-cancer and anti-inflammatory activity [198]. C/EBPβ could also serve as a potential target in

97 reducing MDSCs function as a few inhibitors are available but need to be further evaluated [199, 200].

Taken together, these studies have allowed us to identify HDAC11 as a novel regulator of the tolerogenic and immunosuppressive microenvironment induced by cancer. The dual role of HDAC11 in regulating MDSCs suppressive function and T cell anti-tumor effect reflects the complexity of its function and sheds lights on the therapeutic potential of this novel molecule.

98

Chapter 4: Conclusions and Future Directions.

HDAC11 was first discovered and cloned in 2002 and was characterized as a

small protein with 39 kDa molecule weight and expressed largely different among

different tissues [79]. It shared limited similarity with other HDACs in its family, and was

thus given its own class as Class IV HDACs. Unlike other HDACs in its family, little is

known of HDAC11's function among the scientific community. HDAC11 has the

potential to regulate DNA replication through manipulation of chromatin structures and

alter chromatin accessibility to transcription in a few genes such as cdt1, cdc25A and

oligodendrocyte specific genes MBP and PLP genes [91, 201, 202]. Recently, HDAC11

depletion is believed to cause cancer cell death and inhibit the metabolic activities in a

few cancer cell lines, including colon, prostate, breast and ovarian lines by using a

catalytically impaired variant of HDAC11 [84]. Other HDACs have been shown to

demonstrate an effect on both cancer cells and the host immune system. However, the regulatory role of HDAC11 on the immune system is still undergoing investigation. In order to assess the capability of HDAC11 as a potential therapeutic, a better understanding of its function is urgently needed.

Knocking out HDAC11 in the mouse model is not lethal, at least in the C57BL/6 background. Extensive studies in our group have illuminated a unique role of HDAC11 in the immune system. In our observations, in other experiments performed in the

99 laboratory outside of this thesis, there is no difference in the size of major organs such

as the brain, spleen, liver, thymus and heart from HDAC11KO mouse. In addition,

lymph nodes appear to be slightly larger in the knockout. HDAC11KO animals also

share relatively similar blood counts for monocytes, granulocytes, white blood cells and

lymphocytes, whereas colony forming assay results showed a slight increase of CFU-M,

CFU-G, CFU-GM colonies with a dramatic increase of the CFU-PreB colony (data not

shown in this dissertation). In Chapter 2 we have reviewed the evidence that aging

HDAC11KO mice (18 months old) demonstrated BM hypercellularity with granulocytic

expansion and splenomegaly, possibly resulting from increased extramedullary

hematopoiesis. Additionally, a significant decrease in the viability of neutrophils isolated

from the HDAC11KO mice bone marrow was observed when these cells were

stimulated with GM-CSF compared to WT control, indicating that HDAC11KO

neutrophils have an even shorter life span in response to stimulation. So far we have

uncovered no evidence regarding the long-term survival for HDAC11KO MDSCs.

Using the HDAC11-eGFP transgenic mouse model, previous members in our lab

were able to visually identify the HDAC11 gene expression profile in immune cells. Their

data suggest that HDAC11 expresses at a higher level in the neutrophils/granulocytes,

but remains low in the macrophages/monocytes. Activation of both CD4+ and CD8+ T cells required a decreased expression of HDAC11. While differentiated into MDSCs,

HDAC11 signal was lost compared with immature myeloid cells express the same

surface marker as MDSCs. Tumors can promote multiple mechanisms to induce

immune dysfunction in both mouse and human. The unique expression profile of

100 HDAC11 drove researchers in our group to further investigate the role of this molecule

in different lineage immune cells.

Villagra demonstrated that HDAC11 regulates the chromatin accessibility for

transcriptional regulation in the promoter region of IL-10 in macrophages [80]. Woods

found out that deletion of HDAC11 permits a hyper active phenotype in T cells with an

upregulation of transcription factor T-bet and Eomes [82]. Huang from the Hancock lab

discovered an increased expression of Foxp3 and TGF-β, along with increased

suppressive function in Foxp3+ Tregs [83]. Sahakian published work in identifying

HDAC11KO MDSCs that possessed an enhanced ability to suppress T cell function and promote tumor progression [155]. This dissertation discussed the influence of HDAC11

in neutrophil biology, as well as its role in regulating MDSC function through

transcriptional regulation of C/EBPβ. In collaboration with Sahakian, we found out that

neutrophils lacking HDAC11 gained migratory and phagocytic ability, with an increased

cytokine production profile (TNF-α, IL-6 and IL-1β, data for IL-1β cytokine production is

not listed in this dissertation). Furthermore, the deletion of HDAC11 seems to grant

neutrophils a more active phenotype upon infection. The continuous work carried on

from this MDSCs study was discussed previously in chapter 3. We found out that the

ultra suppressive phenotype of MDSCs lacking HDAC11 was possibly regulated

through transcription factor C/EBPβ, which was proven to be associated with MDSC

suppressive factor Arg1 and Nos2. With an increased level of Arg1 and Nos2, L-

arginine metabolism was increased in HDAC11KO MDSCs, granting those cells

enhanced suppression.

101 Taken all together, we believe that HDAC11 plays a dual yet contradictory role in

the host immune system. Without HDAC11's participation, under pathological conditions

such as cancer or infection, the "immune defenders" such as neutrophils, APCs and T

cells become functionally more active against pathogen or tumor invasion. On the

contrary, "immune suppressors" such as MDSCs become more suppressive.

These findings led us to believe that when targeting HDAC11 as a potential

cancer treatment, the effect of its inhibition on both cancer cells and the immune system

should be taken into consideration to assess its safety and effectiveness. With an

increasing interest in the development of effective HDAC11 inhibitors, more in-depth

investigation should be performed to gain a better understanding of the mechanisms of

HDAC11 that affect the immune system. Data from us and other investigators suggest

that HDAC11 regulates a broad range of genes in different cell types. HDAC11 down-

regulates cytokines such as IL-10, IL-6, TNF-α and IL-1β in matured myeloid cells, while

repressing the expression of transcription factors such as T-bet, Eomes in T cells,

Foxp3 in Tregs and C/EBPβ in MDSCs. Neutrophils lacking HDAC11 displayed a higher

level of migration related genes such as MIP2 and CXCR2. Chromatin immunoprecipitation analysis showed the presence of HDAC11 in the promoters of multiple genes listed above, indicating the potential mechanism of HDAC11 in chromatin remodeling within those genes. Unfortunately, there is no further evidence to help us explain how HDAC11 regulate those genes. Data from Woods and myself did point out that total histone 3 in T cells and Lysine 27 on histone 3 in neutrophils (figure not included in this dissertation) demonstrated an increased acetylation status when lacking HDAC11. However, more experiments in the promoters should be performed to

102 help us understand the true mechanism. Secondly, most members in the HDAC family

are known to function as a protein complex. Villagra had discovered that in APCs,

HDAC11 and HDAC6 could interact in the cytosolic and nuclear compartment [78].

Further investigation of how these two HDACs interact as well as other proteins

potentially associated with this regulatory complex will certainly help us unveil the

secrets of HDAC11. HDAC11 is predominately localized in the nucleus with a limited amount of protein detectable in the cytosolic compartment. It will be necessary to find out the mechanism of how HDAC11 is shuttling in and out of the nucleus in response to

stimulation, which will provide a better insight into its function in immune cells. Last but

not least, more experiments should be designed and performed to identify other

possible pathways for MDSCs function under HDAC11 inhibition. Despite the critical

role of C/EBPβ in regulating MDSC function, multiple pathways have been uncovered in

the past contributing to this suppressive cell type. For instance, STAT3 was shown to

enhance MDSC proliferation through up-regulation of cell cycle related genes such as

Bcl-x1, cyclin D1 and survivin, as well as S100A8 and S100A9 [9, 203, 204]. A better

understanding of the complete regulatory role of HDAC11 in MDSCs could provide

more potential therapeutic targets in combination with HDAC11 inhibition.

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List of references

1. Siegel, R.L., K.D. Miller, and A. Jemal, Cancer Statistics, 2017. CA Cancer J Clin, 2017. 67(1): p. 7- 30. 2. Hanahan, D. and R.A. Weinberg, The hallmarks of cancer. Cell, 2000. 100(1): p. 57-70. 3. Fouad YA1, A.C., Revisiting the hallmarks of cancer. Am J Cancer Res, 2017. 7(5): p. 1016-1036. 4. Dempsey, P.W., S.A. Vaidya, and G. Cheng, The art of war: Innate and adaptive immune responses. Cell Mol Life Sci, 2003. 60(12): p. 2604-21. 5. Iwasaki, A. and R. Medzhitov, Control of adaptive immunity by the innate immune system. Nat Immunol, 2015. 16(4): p. 343-53. 6. Dunn GP1, B.A., Ikeda H, Old LJ, Schreiber RD., Cancer immunoediting: from immunosurveillance to tumor escape. Nature Immunology, 2002. 3(11): p. 991-8. 7. Kumar, V., et al., The Nature of Myeloid-Derived Suppressor Cells in the Tumor Microenvironment. Trends Immunol, 2016. 37(3): p. 208-220. 8. Poschke, I. and R. Kiessling, On the armament and appearances of human myeloid-derived suppressor cells. Clin Immunol, 2012. 144(3): p. 250-68. 9. Cheng, P., et al., Inhibition of dendritic cell differentiation and accumulation of myeloid-derived suppressor cells in cancer is regulated by S100A9 protein. J Exp Med, 2008. 205(10): p. 2235-49. 10. Almand, B., et al., Increased Production of Immature Myeloid Cells in Cancer Patients: A Mechanism of Immunosuppression in Cancer. The Journal of Immunology, 2001. 166(1): p. 678- 689. 11. Song, X., et al., CD11b+/Gr-1+ Immature Myeloid Cells Mediate Suppression of T Cells in Mice Bearing Tumors of IL-1 -Secreting Cells. The Journal of Immunology, 2005. 175(12): p. 8200- 8208. 12. Kroesen M1, G.P., Brok IC2, Armandari I3, Hoogerbrugge PM4, Adema GJ3., HDAC inhibitors and immunotherapy; a double edged sword? oncotarget, 2014. 5(16): p. 6558-72. 13. Scott, A.M., J.D. Wolchok, and L.J. Old, Antibody therapy of cancer. Nat Rev Cancer, 2012. 12(4): p. 278-87. 14. Joyce, J.A. and J.W. Pollard, Microenvironmental regulation of metastasis. Nat Rev Cancer, 2009. 9(4): p. 239-52. 15. Hanahan, D. and L.M. Coussens, Accessories to the crime: functions of cells recruited to the tumor microenvironment. Cancer Cell, 2012. 21(3): p. 309-22. 16. Fridman, W.H., et al., The immune contexture in human tumours: impact on clinical outcome. Nat Rev Cancer, 2012. 12(4): p. 298-306. 17. Shang, B., et al., Prognostic value of tumor-infiltrating FoxP3+ regulatory T cells in cancers: a systematic review and meta-analysis. Sci Rep, 2015. 5: p. 15179. 18. Chang, C., et al., High levels of regulatory T cells in blood are a poor prognostic factor in patients with diffuse large B-cell lymphoma. Am J Clin Pathol, 2015. 144(6): p. 935-44.

104 19. Fozza, C. and M. Longinotti, T-Cell Traffic Jam in Hodgkin's Lymphoma: Pathogenetic and Therapeutic Implications. Adv Hematol, 2011. 2011: p. 501659. 20. Koreishi, A.F., et al., The role of cytotoxic and regulatory T cells in relapsed/refractory Hodgkin lymphoma. Appl Immunohistochem Mol Morphol, 2010. 18(3): p. 206-11. 21. Curiel, T.J., et al., Specific recruitment of regulatory T cells in ovarian carcinoma fosters immune privilege and predicts reduced survival. Nat Med, 2004. 10(9): p. 942-9. 22. Bailey, C., et al., Chemokine expression is associated with the accumulation of tumour associated macrophages (TAMs) and progression in human colorectal cancer. Clin Exp Metastasis, 2007. 24(2): p. 121-30. 23. Harlin, H., et al., Chemokine expression in melanoma metastases associated with CD8+ T-cell recruitment. Cancer Res, 2009. 69(7): p. 3077-85. 24. Hong, M., et al., Chemotherapy induces intratumoral expression of chemokines in cutaneous melanoma, favoring T-cell infiltration and tumor control. Cancer Res, 2011. 71(22): p. 6997- 7009. 25. Buchbinder, E.I. and A. Desai, CTLA-4 and PD-1 Pathways: Similarities, Differences, and Implications of Their Inhibition. Am J Clin Oncol, 2016. 39(1): p. 98-106. 26. Gabrilovich, D.I., S. Ostrand-Rosenberg, and V. Bronte, Coordinated regulation of myeloid cells by tumours. Nat Rev Immunol, 2012. 12(4): p. 253-68. 27. Melani, C., et al., Myeloid cell expansion elicited by the progression of spontaneous mammary carcinomas in c-erbB-2 transgenic BALB/c mice suppresses immune reactivity. Blood, 2003. 102(6): p. 2138-45. 28. Gabrilovich, D.I., et al., Mechanism of Immune Dysfunction in Cancer Mediated by Immature Gr- 1+ Myeloid Cells. The Journal of Immunology, 2001. 166(9): p. 5398-5406. 29. Gabrilovich, D.I., et al., The terminology issue for myeloid-derived suppressor cells. Cancer Res, 2007. 67(1): p. 425; author reply 426. 30. Gabrilovich, D.I. and S. Nagaraj, Myeloid-derived suppressor cells as regulators of the immune system. Nat Rev Immunol, 2009. 9(3): p. 162-74. 31. Marigo, I., et al., Tumor-induced tolerance and immune suppression depend on the C/EBPbeta transcription factor. Immunity, 2010. 32(6): p. 790-802. 32. Solito, S., et al., A human promyelocytic-like population is responsible for the immune suppression mediated by myeloid-derived suppressor cells. Blood, 2011. 118(8): p. 2254-65. 33. Millrud CR1, B.C., Leandersson K1., On the origin of myeloid-derived suppressor cells. Oncotarget, 2017. 8(2): p. 3649-3665. 34. Morales, J.K., et al., GM-CSF is one of the main breast tumor-derived soluble factors involved in the differentiation of CD11b-Gr1- bone marrow progenitor cells into myeloid-derived suppressor cells. Breast Cancer Res Treat, 2010. 123(1): p. 39-49. 35. Peranzoni, E., et al., Myeloid-derived suppressor cell heterogeneity and subset definition. Curr Opin Immunol, 2010. 22(2): p. 238-44. 36. Youn, J.I. and D.I. Gabrilovich, The biology of myeloid-derived suppressor cells: the blessing and the curse of morphological and functional heterogeneity. Eur J Immunol, 2010. 40(11): p. 2969- 75. 37. Bronte V1, W.M., Overwijk WW, Surman DR, Pericle F, Rosenberg SA, Restifo NP., Apoptotic death of CD8+ T lymphocytes after immunization: induction of a suppressive population of Mac- 1+/Gr-1+ cells. Journal of Immunology, 1998. 161(10): p. 5313-20. 38. Movahedi, K., et al., Identification of discrete tumor-induced myeloid-derived suppressor cell subpopulations with distinct T cell-suppressive activity. Blood, 2008. 111(8): p. 4233-44. 39. Meirow, Y., J. Kanterman, and M. Baniyash, Paving the Road to Tumor Development and Spreading: Myeloid-Derived Suppressor Cells are Ruling the Fate. Front Immunol, 2015. 6: p. 523.

105 40. Youn, J.I., et al., Characterization of the nature of granulocytic myeloid-derived suppressor cells in tumor-bearing mice. J Leukoc Biol, 2012. 91(1): p. 167-81. 41. Kusmartsev, S., et al., Antigen-Specific Inhibition of CD8+ T Cell Response by Immature Myeloid Cells in Cancer Is Mediated by Reactive Oxygen Species. The Journal of Immunology, 2004. 172(2): p. 989-999. 42. Youn, J.I., et al., Subsets of Myeloid-Derived Suppressor Cells in Tumor-Bearing Mice. The Journal of Immunology, 2008. 181(8): p. 5791-5802. 43. Dolcetti, L., et al., Hierarchy of immunosuppressive strength among myeloid-derived suppressor cell subsets is determined by GM-CSF. Eur J Immunol, 2010. 40(1): p. 22-35. 44. Foell, D., et al., S100 proteins expressed in phagocytes: a novel group of damage-associated molecular pattern molecules. J Leukoc Biol, 2007. 81(1): p. 28-37. 45. Gallina, G., et al., Tumors induce a subset of inflammatory monocytes with immunosuppressive activity on CD8+ T cells. J Clin Invest, 2006. 116(10): p. 2777-90. 46. Kusmartsev, S. and D.I. Gabrilovich, STAT1 Signaling Regulates Tumor-Associated Macrophage- Mediated T Cell Deletion. The Journal of Immunology, 2005. 174(8): p. 4880-4891. 47. Bronte, V., et al., L-arginine metabolism in myeloid cells controls T-lymphocyte functions. Trends in Immunology, 2003. 24(6): p. 301-305. 48. Rodriguez, P.C., A.C. Ochoa, and A.A. Al-Khami, Arginine Metabolism in Myeloid Cells Shapes Innate and Adaptive Immunity. Front Immunol, 2017. 8: p. 93. 49. Badn, W. and V. Bronte, Myeloid-Derived Suppressor Cells in Cancer. 2012: p. 217-229. 50. Mazzoni, A., et al., Myeloid Suppressor Lines Inhibit T Cell Responses by an NO-Dependent Mechanism. The Journal of Immunology, 2002. 168(2): p. 689-695. 51. Macphail, S.E., et al., Nitric Oxide Regulation of Human Peripheral Blood Mononuclear Cells: Critical Time Dependence and Selectivity for Cytokine versus Chemokine Expression. The Journal of Immunology, 2003. 171(9): p. 4809-4815. 52. Corzo, C.A., et al., Mechanism regulating reactive oxygen species in tumor-induced myeloid- derived suppressor cells. J Immunol, 2009. 182(9): p. 5693-701. 53. Huang, B., et al., Gr-1+CD115+ immature myeloid suppressor cells mediate the development of tumor-induced T regulatory cells and T-cell anergy in tumor-bearing host. Cancer Res, 2006. 66(2): p. 1123-31. 54. Sharma S1, Y.S., Zhu L, Reckamp K, Gardner B, Baratelli F, Huang M, Batra RK, Dubinett SM., Tumor cyclooxygenase-2/prostaglandin E2-dependent promotion of FOXP3 expression and CD4+ CD25+ T regulatory cell activities in lung cancer. Cancer Research, 2006. 65(12): p. 5211-20.

55. Serafini, P., et al., Myeloid-derived suppressor cells promote cross-tolerance in B-cell lymphoma by expanding regulatory T cells. Cancer Res, 2008. 68(13): p. 5439-49.

56. Ugel, S., et al., Tumor-induced myeloid deviation: when myeloid-derived suppressor cells meet tumor-associated macrophages. J Clin Invest, 2015. 125(9): p. 3365-76. 57. De Santo, C., et al., Nitroaspirin corrects immune dysfunction in tumor-bearing hosts and promotes tumor eradication by cancer vaccination. Proc Natl Acad Sci U S A, 2005. 102(11): p. 4185-90. 58. Kusmartsev S1, C.F., Yu B, Nefedova Y, Sotomayor E, Lush R, Gabrilovich D., All-trans-retinoic acid eliminates immature myeloid cells from tumor-bearing mice and improves the effect of vaccination. Cancer Research, 2003. 63(15): p. 4441-4449. 59. Vincent, J., et al., 5-Fluorouracil selectively kills tumor-associated myeloid-derived suppressor cells resulting in enhanced T cell-dependent antitumor immunity. Cancer Res, 2010. 70(8): p. 3052-61.

106 60. Ugel, S., et al., Immune tolerance to tumor antigens occurs in a specialized environment of the spleen. Cell Rep, 2012. 2(3): p. 628-39. 61. Ries, C.H., et al., Targeting tumor-associated macrophages with anti-CSF-1R antibody reveals a strategy for cancer therapy. Cancer Cell, 2014. 25(6): p. 846-59. 62. Martinez, F.O., et al., Transcriptional Profiling of the Human Monocyte-to-Macrophage Differentiation and Polarization: New Molecules and Patterns of Gene Expression. The Journal of Immunology, 2006. 177(10): p. 7303-7311. 63. Biswas SK1, G.L., Paul S, Schioppa T, Saccani A, Sironi M, Bottazzi B, Doni A, Vincenzo B, Pasqualini F, Vago L, Nebuloni M, Mantovani A, Sica A., A distinct and unique transcriptional program expressed by tumor-associated macrophages (defective NF-kappaB and enhanced IRF- 3/STAT1 activation). Blood, 2006. 107(5): p. 2112-22. 64. Savage, N.D.L., et al., Human Anti-Inflammatory Macrophages Induce Foxp3+GITR+CD25+ Regulatory T Cells, Which Suppress via Membrane-Bound TGF -1. The Journal of Immunology, 2008. 181(3): p. 2220-2226. 65. Schlecker, E., et al., Tumor-infiltrating monocytic myeloid-derived suppressor cells mediate CCR5- dependent recruitment of regulatory T cells favoring tumor growth. J Immunol, 2012. 189(12): p. 5602-11. 66. Biswas, S.K. and A. Mantovani, Macrophage plasticity and interaction with lymphocyte subsets: cancer as a paradigm. Nat Immunol, 2010. 11(10): p. 889-96. 67. Tachibana, T., et al., Increased intratumor Valpha24-positive natural killer T cells: a prognostic factor for primary colorectal carcinomas. Clin Cancer Res, 2005. 11(20): p. 7322-7. 68. Milne, K., et al., Systematic analysis of immune infiltrates in high-grade serous ovarian cancer reveals CD20, FoxP3 and TIA-1 as positive prognostic factors. PLoS One, 2009. 4(7): p. e6412. 69. Coronella JA1, T.P., Kingsbury GA, Truong TD, Hays S, Junghans RP., Evidence for an antigen- driven humoral immune response in medullary ductal breast cancer. Cancer Research 2001. 61(21): p. 7889-99.

70. Balkwill, F.R., M. Capasso, and T. Hagemann, The tumor microenvironment at a glance. J Cell Sci, 2012. 125(Pt 23): p. 5591-6. 71. Wang, M., et al., Role of tumor microenvironment in tumorigenesis. J Cancer, 2017. 8(5): p. 761- 773. 72. Sounni, N.E. and A. Noel, Targeting the tumor microenvironment for cancer therapy. Clin Chem, 2013. 59(1): p. 85-93. 73. Ebos, J.M. and R.S. Kerbel, Antiangiogenic therapy: impact on invasion, disease progression, and metastasis. Nat Rev Clin Oncol, 2011. 8(4): p. 210-21. 74. de Ruijter AJ1, v.G.A., Caron HN, Kemp S, van Kuilenburg AB., Histone deacetylases (HDACs): characterization of the classical HDAC family. Biochem J, 2003. 15(370). 75. Falkenberg, K.J. and R.W. Johnstone, Histone deacetylases and their inhibitors in cancer, neurological diseases and immune disorders. Nat Rev Drug Discov, 2014. 13(9): p. 673-91. 76. Bjerling, P., et al., Functional Divergence between Histone Deacetylases in Fission Yeast by Distinct Cellular Localization and In Vivo Specificity. Molecular and Cellular Biology, 2002. 22(7): p. 2170-2181. 77. Yang, X.J. and E. Seto, The Rpd3/Hda1 family of lysine deacetylases: from bacteria and yeast to mice and men. Nat Rev Mol Cell Biol, 2008. 9(3): p. 206-18. 78. Cheng, F., et al., Divergent roles of histone deacetylase 6 (HDAC6) and histone deacetylase 11 (HDAC11) on the transcriptional regulation of IL10 in antigen presenting cells. Mol Immunol, 2014. 60(1): p. 44-53.

107 79. Gao, L., et al., Cloning and functional characterization of HDAC11, a novel member of the human histone deacetylase family. J Biol Chem, 2002. 277(28): p. 25748-55. 80. Villagra, A., et al., The histone deacetylase HDAC11 regulates the expression of interleukin 10 and immune tolerance. Nat Immunol, 2009. 10(1): p. 92-100. 81. Bala, S., et al., Alcohol-induced miR-155 and HDAC11 inhibit negative regulators of the TLR4 pathway and lead to increased LPS responsiveness of Kupffer cells in alcoholic liver disease. J Leukoc Biol, 2017. 102(2): p. 487-498. 82. Woods, D.M., et al., T cells lacking HDAC11 have increased effector functions and mediate enhanced alloreactivity in a murine model. Blood, 2017. 130(2): p. 146-155. 83. Huang, J., et al., Histone/protein deacetylase 11 targeting promotes Foxp3+ Treg function. Sci Rep, 2017. 7(1): p. 8626. 84. Deubzer, H.E., et al., HDAC11 is a novel drug target in carcinomas. Int J Cancer, 2013. 132(9): p. 2200-8. 85. S, B., et al., Interleukin 2 Gene Transcription is Regulated by Ikaros-induced Changes in Histone Acetylation in Anergic T cells. Blood, 2007. 109(7): p. 2878-2886. 86. Wang, W., et al., Histone deacetylase 11 suppresses p53 expression in pituitary tumor cells. Cell Biol Int, 2017. 87. Thole, T.M., et al., Neuroblastoma cells depend on HDAC11 for mitotic cell cycle progression and survival. Cell Death Dis, 2017. 8(3): p. e2635. 88. Wong, P.G., et al., Chromatin unfolding by Cdt1 regulates MCM loading via opposing functions of HBO1 and HDAC11-geminin. Cell Cycle, 2010. 9(21): p. 4351-63. 89. Lozada EM1, A.Z., 2, Yin M1, Redilla N1, Rice K1, Stambrook PJ1., Acetylation and deacetylation of Cdc25A constitutes a novel mechanism for modulating Cdc25A functions with implications for cancer. oncotarget, 2016. 7(15): p. 20425-39. 90. Liu, H., et al., Developmental expression of histone deacetylase 11 in the murine brain. J Neurosci Res, 2008. 86(3): p. 537-43. 91. Liu, H., et al., Histone deacetylase 11 regulates oligodendrocyte-specific gene expression and cell development in OL-1 oligodendroglia cells. Glia, 2009. 57(1): p. 1-12. 92. Toropainen, S., et al., The down-regulation of the human MYC gene by the nuclear hormone 1alpha,25-dihydroxyvitamin D3 is associated with cycling of corepressors and histone deacetylases. J Mol Biol, 2010. 400(3): p. 284-94. 93. Liu FH1, L.S., Li XX2, Wang S2,3, Li MG2, Guan L4, Luan TG4, Liu ZG2, Liu ZJ1, Yang PC2., Vitamin D3 induces vitamin D receptor and HDAC11 binding to relieve the promoter of the tight junction proteins. Oncotarget, 2017. 8(35): p. 58781-58789. 94. Zhang, Z., et al., Quantitation of HDAC1 mRNA expression in invasive carcinoma of the breast*. Breast Cancer Res Treat, 2005. 94(1): p. 11-6. 95. Wilson, A.J., et al., Histone deacetylase 3 (HDAC3) and other class I HDACs regulate colon cell maturation and p21 expression and are deregulated in human colon cancer. J Biol Chem, 2006. 281(19): p. 13548-58. 96. Choi JH1, K.H., Yoon BI, Kim JH, Han SU, Joo HJ, Kim DY., Expression profile of histone deacetylase 1 in gastric cancer tissues. Jpn J Cancer Res. Jpn J Cancer Res, 2001. 92(12): p. 1300- 4. 97. Halkidou, K., et al., Upregulation and nuclear recruitment of HDAC1 in hormone refractory prostate cancer. Prostate, 2004. 59(2): p. 177-89. 98. Huang, B.H., et al., Inhibition of increases apoptosis and p21Cip1/WAF1 expression, independent of histone deacetylase 1. Cell Death Differ, 2005. 12(4): p. 395-404. 99. Zhu P1, M.E., Mengwasser J, Schlag P, Janssen KP, Göttlicher M., Induction of HDAC2 expression upon loss of APC in colorectal tumorigenesis. . cancer cell, 2004. 5(5): p. 455-63.

108 100. Zhang Z1, Y.H., Toyama T, Sugiura H, Omoto Y, Ando Y, Mita K, Hamaguchi M, Hayashi S, Iwase H., HDAC6 expression is correlated with better survival in breast cancer. . Clinical cancer research, 2004. 10(20): p. 6962-8. 101. Dinarello, C.A., Anti-inflammatory Agents: Present and Future. Cell, 2010. 140(6): p. 935-50. 102. Woan, K.V., et al., Modulation of antigen-presenting cells by HDAC inhibitors: implications in autoimmunity and cancer. Immunol Cell Biol, 2012. 90(1): p. 55-65. 103. Bolden, J.E., M.J. Peart, and R.W. Johnstone, Anticancer activities of histone deacetylase inhibitors. Nat Rev Drug Discov, 2006. 5(9): p. 769-84. 104. Elvir, L., et al., Epigenetic Regulation of Motivated Behaviors by Histone Deacetylase Inhibitors. Neurosci Biobehav Rev, 2017. 105. Marks, P.A. and R. Breslow, Dimethyl sulfoxide to vorinostat: development of this histone deacetylase inhibitor as an anticancer drug. Nat Biotechnol, 2007. 25(1): p. 84-90. 106. Prince, H.M. and M. Dickinson, Romidepsin for cutaneous T-cell lymphoma. Clin Cancer Res, 2012. 18(13): p. 3509-15. 107. Mottamal, M., et al., Histone deacetylase inhibitors in clinical studies as templates for new anticancer agents. Molecules, 2015. 20(3): p. 3898-941. 108. Maeda, T., et al., Up-regulation of costimulatory/adhesion molecules by histone deacetylase inhibitors in acute myeloid leukemia cells. Blood, 2000. 96(12): p. 3847-56. 109. Magner, W.J., et al., Activation of MHC class I, II, and CD40 gene expression by histone deacetylase inhibitors. J Immunol, 2000. 165(12): p. 7017-24. 110. Armeanu, S., et al., Natural killer cell-mediated lysis of hepatoma cells via specific induction of NKG2D ligands by the histone deacetylase inhibitor sodium valproate. Cancer Res, 2005. 65(14): p. 6321-9. 111. Skov, S., et al., Cancer cells become susceptible to natural killer cell killing after exposure to histone deacetylase inhibitors due to glycogen synthase kinase-3-dependent expression of MHC class I-related chain A and B. Cancer Res, 2005. 65(23): p. 11136-45. 112. Khan, A.N., W.J. Magner, and T.B. Tomasi, An epigenetic vaccine model active in the prevention and treatment of melanoma. J Transl Med, 2007. 5: p. 64. 113. Reddy, P., et al., Histone deacetylase inhibitor suberoylanilide hydroxamic acid reduces acute graft-versus-host disease and preserves graft-versus-leukemia effect. Proc Natl Acad Sci U S A, 2004. 101(11): p. 3921-6. 114. Nicolas-Avila, J.A., J.M. Adrover, and A. Hidalgo, Neutrophils in Homeostasis, Immunity, and Cancer. Immunity, 2017. 46(1): p. 15-28. 115. Phillipson, M. and P. Kubes, The neutrophil in vascular inflammation. Nat Med, 2011. 17(11): p. 1381-90. 116. Kruger, P., et al., Neutrophils: Between host defence, immune modulation, and tissue injury. PLoS Pathog, 2015. 11(3): p. e1004651. 117. Summers, C., et al., Neutrophil kinetics in health and disease. Trends Immunol, 2010. 31(8): p. 318-24. 118. Furze, R.C. and S.M. Rankin, Neutrophil mobilization and clearance in the bone marrow. Immunology, 2008. 125(3): p. 281-8. 119. Templeton, A.J., et al., Prognostic role of neutrophil-to-lymphocyte ratio in solid tumors: a systematic review and meta-analysis. J Natl Cancer Inst, 2014. 106(6): p. dju124. 120. Gentles, A.J., et al., The prognostic landscape of genes and infiltrating immune cells across human cancers. Nat Med, 2015. 21(8): p. 938-945. 121. Borregaard, N., Neutrophils, from marrow to microbes. Immunity, 2010. 33(5): p. 657-70. 122. Mayadas, T.N., X. Cullere, and C.A. Lowell, The multifaceted functions of neutrophils. Annu Rev Pathol, 2014. 9: p. 181-218.

109 123. Pillay, J., et al., In vivo labeling with 2H2O reveals a human neutrophil lifespan of 5.4 days. Blood, 2010. 116(4): p. 625-7. 124. Mantovani, A., et al., Neutrophils in the activation and regulation of innate and adaptive immunity. Nat Rev Immunol, 2011. 11(8): p. 519-31. 125. Kolaczkowska, E. and P. Kubes, Neutrophil recruitment and function in health and inflammation. Nat Rev Immunol, 2013. 13(3): p. 159-75. 126. Cerutti, A., I. Puga, and G. Magri, The B cell helper side of neutrophils. J Leukoc Biol, 2013. 94(4): p. 677-82. 127. Kalyan, S. and D. Kabelitz, When neutrophils meet T cells: beginnings of a tumultuous relationship with underappreciated potential. Eur J Immunol, 2014. 44(3): p. 627-33. 128. Takashima, A. and Y. Yao, Neutrophil plasticity: acquisition of phenotype and functionality of antigen-presenting cell. J Leukoc Biol, 2015. 98(4): p. 489-96. 129. Ruffell, B., N.I. Affara, and L.M. Coussens, Differential macrophage programming in the tumor microenvironment. Trends Immunol, 2012. 33(3): p. 119-26. 130. Coffelt, S.B., M.D. Wellenstein, and K.E. de Visser, Neutrophils in cancer: neutral no more. Nat Rev Cancer, 2016. 16(7): p. 431-46. 131. Galdiero, M.R., et al., Tumor associated macrophages and neutrophils in cancer. Immunobiology, 2013. 218(11): p. 1402-10. 132. Galli, S.J., N. Borregaard, and T.A. Wynn, Phenotypic and functional plasticity of cells of innate immunity: macrophages, mast cells and neutrophils. Nat Immunol, 2011. 12(11): p. 1035-44. 133. Fridlender, Z.G., et al., Polarization of tumor-associated neutrophil phenotype by TGF-beta: "N1" versus "N2" TAN. Cancer Cell, 2009. 16(3): p. 183-94. 134. Nozawa, H., C. Chiu, and D. Hanahan, Infiltrating neutrophils mediate the initial angiogenic switch in a mouse model of multistage carcinogenesis. Proceedings of the National Academy of Sciences of the United States of America, 2006. 103(33): p. 12493-12498. 135. Murray, P.J., et al., Macrophage activation and polarization: nomenclature and experimental guidelines. Immunity, 2014. 41(1): p. 14-20. 136. Brinkmann, V. and A. Zychlinsky, Neutrophil extracellular traps: is immunity the second function of chromatin? J Cell Biol, 2012. 198(5): p. 773-83. 137. Branzk, N., et al., Neutrophils sense microbe size and selectively release neutrophil extracellular traps in response to large pathogens. Nat Immunol, 2014. 15(11): p. 1017-25. 138. Brinkmann V1, R.U., Goosmann C, Fauler B, Uhlemann Y, Weiss DS, Weinrauch Y, Zychlinsky A., Neutrophil extracellular traps kill bacteria. Science, 2004. 303(5663): p. 1532-5. 139. Caudrillier, A., et al., Platelets induce neutrophil extracellular traps in transfusion-related acute lung injury. J Clin Invest, 2012. 122(7): p. 2661-71. 140. Carestia, A., et al., Mediators and molecular pathways involved in the regulation of neutrophil extracellular trap formation mediated by activated platelets. J Leukoc Biol, 2016. 99(1): p. 153- 62. 141. Fuchs, T.A., et al., Novel cell death program leads to neutrophil extracellular traps. J Cell Biol, 2007. 176(2): p. 231-41. 142. Radic, M., Clearance of Apoptotic Bodies, NETs, and Biofilm DNA: Implications for Autoimmunity. Front Immunol, 2014. 5: p. 365. 143. Wright, H.L., et al., Neutrophil function in inflammation and inflammatory diseases. Rheumatology (Oxford), 2010. 49(9): p. 1618-31. 144. Mantovani, A., et al., Cancer-related inflammation. Nature, 2008. 454(7203): p. 436-44. 145. Fiedler, K. and C. Brunner, The role of transcription factors in the guidance of granulopoiesis. Am J Blood Res, 2012. 1(2): p. 57-65.

110 146. Goyama, S. and T. Kitamura, Epigenetics in normal and malignant hematopoiesis: An overview and update 2017. Cancer Sci, 2017. 108(4): p. 553-562. 147. Ostuni, R., et al., Epigenetic regulation of neutrophil development and function. Semin Immunol, 2016. 28(2): p. 83-93. 148. Rice, K.L., I. Hormaeche, and J.D. Licht, Epigenetic regulation of normal and malignant hematopoiesis. Oncogene, 2007. 26(47): p. 6697-714. 149. Prasad, P., et al., High-throughput transcription profiling identifies putative epigenetic regulators of hematopoiesis. Blood, 2014. 123(17): p. e46-57. 150. Rosmarin, A.G., Z. Yang, and K.K. Resendes, Transcriptional regulation in myelopoiesis: Hematopoietic fate choice, myeloid differentiation, and leukemogenesis. Exp Hematol, 2005. 33(2): p. 131-43. 151. De Felice L1, T.C., Mascolo MG, Gregorj C, Agostini F, Fiorini R, Gelmetti V, Pascale S, Padula F, Petrucci MT, Arcese W, Nervi C., Histone deacetylase inhibitor valproic acid enhances the cytokine-induced expansion of human hematopoietic stem cells. Cancer Res Treat, 2005. 65(4): p. 1505-13. 152. Bartels, M., et al., Histone deacetylase inhibition modulates cell fate decisions during myeloid differentiation. Haematologica, 2010. 95(7): p. 1052-60. 153. Zhang, X., J.P. Edwards, and D.M. Mosser, Dynamic and Transient Remodeling of the Macrophage IL-10 Promoter during Transcription. The Journal of Immunology, 2006. 177(2): p. 1282-1288. 154. Yao, Y., et al., Interleukin (IL)-4 inhibits IL-10 to promote IL-12 production by dendritic cells. J Exp Med, 2005. 201(12): p. 1899-903. 155. Sahakian, E., et al., Histone deacetylase 11: A novel epigenetic regulator of myeloid derived suppressor cell expansion and function. Mol Immunol, 2015. 63(2): p. 579-85. 156. Heintz, N., BAC to the future: the use of bac transgenic mice for neuroscience research. Nat Rev Neurosci, 2001. 2(12): p. 861-70. 157. MW, P., A new mathematical model for relative quantification in real-time RT-PCR. Nucleic Acids Res, 2001. 1(29): p. e45. 158. Shakespear, M.R., et al., Histone deacetylases as regulators of inflammation and immunity. Trends Immunol, 2011. 32(7): p. 335-43. 159. Angus DC1, L.-Z.W., Lidicker J, Clermont G, Carcillo J, Pinsky MR., Epidemiology of severe sepsis in the United States: Analysis of incidence, outcome, and associated costs of care. Crit Care Med, 2001. 29(7): p. 1303-10. 160. Warren, H.S., Editorial: Mouse models to study sepsis syndrome in humans. J Leukoc Biol, 2009. 86(2): p. 199-201. 161. McCuskey RS, M.P., Urbaschek R, Urbaschek B., Species differences in Kupffer cells and endotoxin sensitivity. Infect Immun., 1984. 45(1): p. 278-80. 162. Maestre, Z., et al., Ecotoxicity assessment of river sediments and a critical evaluation of some of the procedures used in the aquatic oligochaete Tubifex tubifex chronic bioassay. Arch Environ Contam Toxicol, 2007. 53(4): p. 559-70. 163. Avellino, R. and R. Delwel, Expression and regulation of C/EBPalpha in normal myelopoiesis and in malignant transformation. Blood, 2017. 129(15): p. 2083-2091. 164. Sadeghi, K., et al., GM-CSF Down-Regulates TLR Expression via the Transcription Factor PU.1 in Human Monocytes. PLoS One, 2016. 11(10): p. e0162667. 165. Rogers, J.H., et al., E2A Antagonizes PU.1 Activity through Inhibition of DNA Binding. Biomed Res Int, 2016. 2016: p. 3983686. 166. Villagra, A., E.M. Sotomayor, and E. Seto, Histone deacetylases and the immunological network: implications in cancer and inflammation. Oncogene, 2010. 29(2): p. 157-73.

111 167. Zeng, L., et al., HDAC3 is crucial in shear- and VEGF-induced stem cell differentiation toward endothelial cells. J Cell Biol, 2006. 174(7): p. 1059-69. 168. Baylin, S.B. and J.E. Ohm, Epigenetic gene silencing in cancer - a mechanism for early oncogenic pathway ? Nat Rev Cancer, 2006. 6(2): p. 107-16. 169. Wang, Y., et al., Histone hypercitrullination mediates chromatin decondensation and neutrophil extracellular trap formation. J Cell Biol, 2009. 184(2): p. 205-13. 170. Dear, A.E., Epigenetic Modulators and the New Immunotherapies. N Engl J Med, 2016. 374(7): p. 684-6. 171. Shen, L., A. Orillion, and R. Pili, Histone deacetylase inhibitors as immunomodulators in cancer therapeutics. Epigenomics, 2016. 8(3): p. 415-28. 172. Stammler, D., et al., Inhibition of Histone Deacetylases Permits Lipopolysaccharide-Mediated Secretion of Bioactive IL-1beta via a Caspase-1-Independent Mechanism. J Immunol, 2015. 195(11): p. 5421-31. 173. Joshi, P., et al., The functional interactome landscape of the human histone deacetylase family. Mol Syst Biol, 2013. 9: p. 672. 174. Nemzek JA1, H.K., Opp MR, Modeling sepsis in the laboratory: merging sound science with animal well-being. Comp Med, 2008. 58(2): p. 120-8. 175. Ciarlo, E., A. Savva, and T. Roger, Epigenetics in sepsis: targeting histone deacetylases. Int J Antimicrob Agents, 2013. 42 Suppl: p. S8-12. 176. Adcock, I.M., HDAC inhibitors as anti-inflammatory agents. Br J Pharmacol, 2007. 150(7): p. 829- 31. 177. Hicks, A.M., et al., Transferable anticancer innate immunity in spontaneous regression/complete resistance mice. Proc Natl Acad Sci U S A, 2006. 103(20): p. 7753-8. 178. Di Carlo E1, F.G., Lollini P, Colombo MP, Modesti A, Musiani P, The intriguing role of polymorphonuclear neutrophils in antitumor reactions. Blood, 2001. 97(2): p. 339-45. 179. Sagiv, J.Y., et al., Phenotypic diversity and plasticity in circulating neutrophil subpopulations in cancer. Cell Rep, 2015. 10(4): p. 562-73. 180. Wang, R.F., Regulatory T cells and innate immune regulation in tumor immunity. Springer Semin Immunopathol, 2006. 28(1): p. 17-23. 181. Nagaraj, S., et al., Mechanism of T cell tolerance induced by myeloid-derived suppressor cells. J Immunol, 2010. 184(6): p. 3106-16. 182. Bronte, V. and P. Zanovello, Regulation of immune responses by L-arginine metabolism. Nat Rev Immunol, 2005. 5(8): p. 641-54. 183. Ostrand-Rosenberg, S., Myeloid-derived suppressor cells: more mechanisms for inhibiting antitumor immunity. Cancer Immunol Immunother, 2010. 59(10): p. 1593-600. 184. Grabiec, A.M. and J. Potempa, Epigenetic regulation in bacterial infections: targeting histone deacetylases. Crit Rev Microbiol, 2017: p. 1-15. 185. Sahakian, E., et al., Essential role for histone deacetylase 11 (HDAC11) in neutrophil biology. J Leukoc Biol, 2017. 102(2): p. 475-486. 186. He, Y., et al., Effects of Adoptive Transferring Different Sources of Myeloid-Derived Suppressor Cells in Mice Corneal Transplant Survival. Transplantation, 2015. 99(10): p. 2102-8. 187. Hirai, H., et al., C/EBPbeta is required for 'emergency' granulopoiesis. Nat Immunol, 2006. 7(7): p. 732-9. 188. Gupta AK1, K.B., CCAAT/enhancer binding protein-beta trans-activates murine nitric oxide synthase 2 gene in an MTAL cell line. Am J Physiol Renal Physiol, 1999. 276(4 pt 2): p. F599-605. 189. Pauleau, A.L., et al., Enhancer-Mediated Control of Macrophage-Specific Arginase I Expression. The Journal of Immunology, 2004. 172(12): p. 7565-7573.

112 190. Teng, X., C/EBP-beta Mediates iNOS Induction by Hypoxia in Rat Pulmonary Microvascular Smooth Muscle Cells. Circulation Research, 2001. 90(2): p. 125-127. 191. Bingisser RM1, T.P., Holt PG, Kees UR., Macrophage-derived nitric oxide regulates T cell activation via reversible disruption of the Jak3/STAT5 signaling pathway. Journal of Immunology, 1998. 160(12): p. 5729-34. 192. Serafini P1, C.R., Noonan KA, Tan G, Bronte V, Borrello I., High-dose granulocyte-macrophage colony-stimulating factor-producing vaccines impair the immune response through the recruitment of myeloid suppressor cells. Cancer Research, 2004. 64(17): p. 6337-43. 193. Rath, M., et al., Metabolism via Arginase or Nitric Oxide Synthase: Two Competing Arginine Pathways in Macrophages. Front Immunol, 2014. 5: p. 532. 194. Rodriguez, P.C. and A.C. Ochoa, Arginine regulation by myeloid derived suppressor cells and tolerance in cancer: mechanisms and therapeutic perspectives. Immunol Rev, 2008. 222: p. 180- 91. 195. Fournel, M., et al., MGCD0103, a novel isotype-selective histone deacetylase inhibitor, has broad spectrum antitumor activity in vitro and in vivo. Mol Cancer Ther, 2008. 7(4): p. 759-68. 196. Gargett, T., et al., GM-CSF signalling blockade and chemotherapeutic agents act in concert to inhibit the function of myeloid-derived suppressor cells in vitro. Clin Transl Immunology, 2016. 5(12): p. e119. 197. Holmgaard, R.B., et al., Targeting myeloid-derived suppressor cells with colony stimulating factor-1 receptor blockade can reverse immune resistance to immunotherapy in indoleamine 2,3- dioxygenase-expressing tumors. EBioMedicine, 2016. 6: p. 50-58. 198. Probst, B.L., et al., RTA 408, A Novel Synthetic Triterpenoid with Broad Anticancer and Anti- Inflammatory Activity. PLoS One, 2015. 10(4): p. e0122942. 199. Harmon AW1, P.Y., Harp JB., Genistein inhibits CCAAT/enhancer-binding protein beta (C/EBPbeta) activity and 3T3-L1 adipogenesis by increasing C/EBP homologous protein expression. Biochem J., 2002. 367(pt 1): p. 203-8. 200. Rochford, J.J., et al., ETO/MTG8 is an inhibitor of C/EBPbeta activity and a regulator of early adipogenesis. Mol Cell Biol, 2004. 24(22): p. 9863-72. 201. Glozak, M.A. and E. Seto, Acetylation/deacetylation modulates the stability of DNA replication licensing factor Cdt1. J Biol Chem, 2009. 284(17): p. 11446-53. 202. Byun, S.K., et al., HDAC11 Inhibits Myoblast Differentiation through Repression of MyoD- Dependent Transcription. Mol Cells, 2017. 40(9): p. 667-676. 203. Yu, H., D. Pardoll, and R. Jove, STATs in cancer inflammation and immunity: a leading role for STAT3. Nat Rev Cancer, 2009. 9(11): p. 798-809. 204. Poschke, I., et al., Immature immunosuppressive CD14+HLA-DR-/low cells in melanoma patients are Stat3hi and overexpress CD80, CD83, and DC-sign. Cancer Res, 2010. 70(11): p. 4335-45.

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