LIMP-2 Expression Is Critical for Β-Glucocerebrosidase Activity and Α-Synuclein Clearance
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Serum Level of Cathepsin B and Its Density in Men with Prostate Cancer As Novel Markers of Disease Progression
ANTICANCER RESEARCH 24: 2573-2578 (2004) Serum Level of Cathepsin B and its Density in Men with Prostate Cancer as Novel Markers of Disease Progression HIDEAKI MIYAKE1, ISAO HARA2 and HIROSHI ETO1 1Department of Urology, Hyogo Medical Center for Adults, 13-70 Kitaohji-cho, Akashi; 2Department of Urology, Kobe University School of Medicine, 7-5-1 Kusunoki-cho, Kobe, Japan Abstract. Background: Cathepsin B has been shown to play in men in Western industrialized countries and is the second an important role in invasion and metastasis of prostate leading cause of cancer-related death (1). Recent cancer. The objective of this study was to determine whether progression in the fields of diagnosis and follow-up using serum levels of cathepsin B and its density (cathepsin B-D) prostate-specific antigen (PSA) and its associated could be used as predictors of disease extension as well as parameters have contributed to early detection and accurate prognosis in patients with prostate cancer. Materials and prediction of prognosis (2, 3). However, despite these Methods: Serum levels of cathepsin B in 60 healthy controls, advances, more than 50% of patients still show evidence of 80 patients with benign prostatic hypertrophy (BPH) and 120 advanced disease at the time of diagnosis, and the currently patients with prostate cancer were measured by a sandwich available parameters are not correlated with the clinical enzyme immunoassay. Cathepsin B-D was calculated by course in some patients during progression to hormone- dividing the serum levels of cathepsin B by the prostate refractory disease (4); hence, there is a pressing need to volume, which was measured using transrectal ultra- develop a novel diagnostic and monitoring marker system sonography. -
And Peptide Sequences (>95 % Confidence) in the Non-Raft Fraction
Supplementary Table 1: Protein identities, their probability scores (protein score and expect score) and peptide sequences (>95 % confidence) in the non-raft fraction. Protein name Protein score Expect score Peptide sequences Glial fibrillary acidic protein 97 0.000058 LALDIEIATYR 0.0000026 LALDIEIATYR Phosphatidylinositol 3-kinase 25 0.038 HGDDLR Uveal autoantigen with coiled-coil domains and ankyrin repeats protein 30 0.03 TEELNR ADAM metallopeptidase domain 32 347 0.0072 SGSICDK 0.0059 YTFCPWR 0.00028 CSEVGPYINR 0.0073 DSASVIYAFVR 0.00044 DSASVIYAFVR 0.00047 LICTYPLQTPFLR 0.0039 LICTYPLQTPFLR 0.001 LICTYPLQTPFLR 0.0000038 AYCFDGGCQDIDAR 0.00000043 AYCFDGGCQDIDAR 0.0000042 VNQCSAQCGGNGVCTSR 0.0000048 NAPFACYEEIQSQTDR 0.000019 NAPFACYEEIQSQTDR Alpha-fetoprotein 24 0.0093 YIYEIAR Junction plakoglobin 214 0.011 ATIGLIR 0.0038 LVQLLVK 0.000043 EGLLAIFK 0.00027 QEGLESVLK 0.000085 TMQNTSDLDTAR 0.000046 ALMGSPQLVAAVVR 0.01 LLNQPNQWPLVK 0.01 NEGTATYAAAVLFR 0.004 NLALCPANHAPLQEAAVIPR 0.0007 VLSVCPSNKPAIVEAGGMQALGK 0.00086 ILVNQLSVDDVNVLTCATGTLSNLTCNNSK Catenin beta-1 54 0.000043 EGLLAIFK Lysozyme 89 0.0026 STDYGIFQINSR 0.00051 STDYGIFQINSR 1 0.0000052 STDYGIFQINSR Annexin A2 72 0.0036 QDIAFAYQR 72 0.0000043 TNQELQEINR Actin, cytoplasmic 61 0.023 IIAPPER 0.021 IIAPPER 0.0044 AGFAGDDAPR 0.013 EITALAPSTMK 0.0013 LCYVALDFEQEMATAASSSSLEK 0.023 IIAPPER 0.021 IIAPPER 0.0044 AGFAGDDAPR 0.0013 LCYVALDFEQEMATAASSSSLEK 0.023 IIAPPER 0.021 IIAPPER 0.0044 AGFAGDDAPR 0.013 EITALAPSTMK ACTA2 protein 35 0.0044 AGFAGDDAPR 0.013 EITALAPSTMK Similar to beta actin -
Hexosaminidase in Mast Β Primary Role of Degranulation Indicator in Mast Cells?
Does β-Hexosaminidase Function Only as a Degranulation Indicator in Mast Cells? The Primary Role of β-Hexosaminidase in Mast Cell Granules This information is current as of September 25, 2021. Nobuyuki Fukuishi, Shinya Murakami, Akane Ohno, Naoya Yamanaka, Nobuaki Matsui, Kenji Fukutsuji, Sakuo Yamada, Kouji Itoh and Masaaki Akagi J Immunol 2014; 193:1886-1894; Prepublished online 11 July 2014; Downloaded from doi: 10.4049/jimmunol.1302520 http://www.jimmunol.org/content/193/4/1886 http://www.jimmunol.org/ Supplementary http://www.jimmunol.org/content/suppl/2014/07/11/jimmunol.130252 Material 0.DCSupplemental References This article cites 52 articles, 18 of which you can access for free at: http://www.jimmunol.org/content/193/4/1886.full#ref-list-1 Why The JI? Submit online. by guest on September 25, 2021 • Rapid Reviews! 30 days* from submission to initial decision • No Triage! Every submission reviewed by practicing scientists • Fast Publication! 4 weeks from acceptance to publication *average Subscription Information about subscribing to The Journal of Immunology is online at: http://jimmunol.org/subscription Permissions Submit copyright permission requests at: http://www.aai.org/About/Publications/JI/copyright.html Email Alerts Receive free email-alerts when new articles cite this article. Sign up at: http://jimmunol.org/alerts The Journal of Immunology is published twice each month by The American Association of Immunologists, Inc., 1451 Rockville Pike, Suite 650, Rockville, MD 20852 Copyright © 2014 by The American Association of -
Quality Assessment Enzyme Analysis for Lysosomal Storage Diseases ERNDIM / EUGT Meeting 5-6 October 2006, Prague
QQuality Assessment Enzyme Analysis for Lysosomal Storage Diseases ERNDIM / EUGT meeting 5-6 October 2006, Prague Results of 1st “large scale” pilot Quality Assessment Enzyme Analysis for Lysosomal Storage Diseases Laboratories Rotterdam Hamburg/Heidelberg Dr, Zoltan Lukacs/Dr. Friederike Bürger QA-pilot for LSD’s the aims • Inter-laboratory variation ─ Many participants: > 30 • Intra-laboratory variation ─ Analyse two pairs of identical control samples (blind) • Proficiency testing of enzyme deficiencies ─ Which samples are best? - Blood samples: most widely used, but impractible - Fibroblasts: good but laborious - EBV lymphoblasts: easy to obtain, not widely used QA-pilot for LSD’s the set up • Samples, without clinical information ─ Leukocytes ─ EBV lymphoblasts ─ Fibroblasts • Enzymes: easy ones ─ 4MU-substrates ─ Simple colorimetric assays • Shipping: economic ─ Send at room temp., postal service - Lyophilised enzymes, stable at room temp. for 5 days - Fibroblasts • Data entry through existing ERNDIM programmes ─ “Metabolite presentation” (www.erndimqa.nl Æ Lysosomal Enzymes) QA-pilot for LSD’s the participants • Questionnaire to members (85 from 21 countries) ─ 40 labs want to join a QA-pilot ─ 65% want to include DBS in future QA-schemes • Samples sent, data returned ─ 40 labs received samples ─ 36 labs entered data on www.erndimqa.nl QA-pilot for LSD’s the samples • 10 samples ─ 4 leukocytes (two duplicate samples) ─ 4 EBV lymphoblasts ─ 2 fibroblasts • 10 easy enzymes ─ specific enzyme activity (e.g.. nmol/h/mg) ─ normalise to -
Acteoside from Ligustrum Robustum (Roxb.) Blume Ameliorates Lipid Metabolism and Synthesis in a Hepg2 Cell Model of Lipid Accumulation
ORIGINAL RESEARCH published: 24 May 2019 doi: 10.3389/fphar.2019.00602 Acteoside From Ligustrum robustum (Roxb.) Blume Ameliorates Lipid Metabolism and Synthesis in a HepG2 Cell Model of Lipid Accumulation Le Sun 1,2†, Fan Yu 1,2†, Fan Yi 3, Lijia Xu 1,2*, Baoping Jiang 1,2*, Liang Le 1,2 and Peigen Xiao 1,2 1 Institute of Medicinal Plant Development, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China, 2 Key Laboratory of Bioactive Substances and Resources Utilization of Chinese Herbal Medicine, Ministry of Education, Beijing, China, 3 Key Laboratory of Cosmetics, China National Light Industry, Beijing Technology and Business Edited by: University, Beijing, China Min Ye, Peking University, China We aimed to ascertain the mechanism underlying the effects of acteoside (ACT) from Reviewer: Wei Song, Ligustrum robustum (Roxb.) Blume (Oleaceae) on lipid metabolism and synthesis. ACT, Peking Union Medical College a water-soluble phenylpropanoid glycoside, is the most abundant and major active Hospital (CAMS), component of L. robustum; the leaves of L. robustum, known as kudingcha (bitter tea), China Shuai Ji, have long been used in China as an herbal tea for weight loss. Recently, based on previous Xuzhou Medical University, studies, our team reached a preliminary conclusion that phenylpropanoid glycosides from China L. robustum most likely contribute substantially to reducing lipid levels, but the mechanism *Correspondence: Lijia Xu remains unclear. Here, we conducted an in silico screen of currently known phenylethanoid [email protected] glycosides from L. robustum and attempted to explore the hypolipidemic mechanism of Baoping Jiang ACT, the representative component of phenylethanoid glycosides in L. -
Oral Ambroxol Increases Brain Glucocerebrosidase Activity in a Nonhuman Primate
Received: 17 November 2016 | Revised: 24 January 2017 | Accepted: 12 February 2017 DOI 10.1002/syn.21967 SHORT COMMUNICATION Oral ambroxol increases brain glucocerebrosidase activity in a nonhuman primate Anna Migdalska-Richards1 | Wai Kin D. Ko2 | Qin Li2,3 | Erwan Bezard2,3,4,5 | Anthony H. V. Schapira1 1Department of Clinical Neurosciences, Institute of Neurology, University College Abstract London, NW3 2PF, United Kingdom Mutations in the glucocerebrosidase 1 (GBA1) gene are related to both Parkinson disease (PD) and 2Motac Neuroscience, Manchester, United Gaucher disease (GD). In both cases, the condition is associated with deficiency of glucocerebrosi- Kingdom dase (GCase), the enzyme encoded by GBA1. Ambroxol is a small molecule chaperone that has 3 Institute of Laboratory Animal Sciences, been shown in mice to cross the blood-brain barrier, increase GCase activity and reduce alpha- China Academy of Medical Sciences, Beijing synuclein protein levels. In this study, we analyze the effect of ambroxol treatment on GCase City, People’s Republic of China activity in healthy nonhuman primates. We show that daily administration of ambroxol results in 4University de Bordeaux, Institut des Maladies Neurodeg en eratives, UMR 5293, increased brain GCase activity. Our work further indicates that ambroxol should be investigated as Bordeaux F-33000, France a novel therapy for both PD and neuronopathic GD in humans. 5CNRS, Institut des Maladies Neurodeg en eratives, UMR 5293, Bordeaux F-33000, France KEYWORDS Correspondence ambroxol, glucocerebrosidase, -
A Computational Approach for Defining a Signature of Β-Cell Golgi Stress in Diabetes Mellitus
Page 1 of 781 Diabetes A Computational Approach for Defining a Signature of β-Cell Golgi Stress in Diabetes Mellitus Robert N. Bone1,6,7, Olufunmilola Oyebamiji2, Sayali Talware2, Sharmila Selvaraj2, Preethi Krishnan3,6, Farooq Syed1,6,7, Huanmei Wu2, Carmella Evans-Molina 1,3,4,5,6,7,8* Departments of 1Pediatrics, 3Medicine, 4Anatomy, Cell Biology & Physiology, 5Biochemistry & Molecular Biology, the 6Center for Diabetes & Metabolic Diseases, and the 7Herman B. Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, IN 46202; 2Department of BioHealth Informatics, Indiana University-Purdue University Indianapolis, Indianapolis, IN, 46202; 8Roudebush VA Medical Center, Indianapolis, IN 46202. *Corresponding Author(s): Carmella Evans-Molina, MD, PhD ([email protected]) Indiana University School of Medicine, 635 Barnhill Drive, MS 2031A, Indianapolis, IN 46202, Telephone: (317) 274-4145, Fax (317) 274-4107 Running Title: Golgi Stress Response in Diabetes Word Count: 4358 Number of Figures: 6 Keywords: Golgi apparatus stress, Islets, β cell, Type 1 diabetes, Type 2 diabetes 1 Diabetes Publish Ahead of Print, published online August 20, 2020 Diabetes Page 2 of 781 ABSTRACT The Golgi apparatus (GA) is an important site of insulin processing and granule maturation, but whether GA organelle dysfunction and GA stress are present in the diabetic β-cell has not been tested. We utilized an informatics-based approach to develop a transcriptional signature of β-cell GA stress using existing RNA sequencing and microarray datasets generated using human islets from donors with diabetes and islets where type 1(T1D) and type 2 diabetes (T2D) had been modeled ex vivo. To narrow our results to GA-specific genes, we applied a filter set of 1,030 genes accepted as GA associated. -
Functional Characterization of Carbohydrate-Active Enzymes from Marine Bacteria
Functional characterization of carbohydrate-active enzymes from marine bacteria I n a u g u r a l d i s s e r t a t i o n zur Erlangung des akademischen Grades eines Doktors der Naturwissenschaften (Dr. rer. nat.) der Mathematisch-Naturwissenschaftlichen Fakultät der Universität Greifswald vorgelegt von Marcus Bäumgen Greifswald, 28.02.2020 Dekan: Prof. Dr. Werner Weitschies 1. Gutachter: Prof. Dr. Uwe T. Bornscheuer 2. Gutachter: Prof. Dr. Harry Brumer Tag der Promotion: 24.06.2020 II III Wissenschaft ist das Werkzeug, welches es uns ermöglicht, das große Puzzel der Natur und des Lebens zu lösen. IV Auch wenn wir den Weg des Wissens und der Weisheit niemals bis zum Ende beschreiten können, so ist doch jeder Schritt, den wir tun, ein Schritt in eine bessere Welt. V Content Abbreviations ..................................................................................................................... IX 1. Introduction ..................................................................................................................... 1 1.1 The marine carbon cycle .............................................................................................. 1 1.1.1 Algal blooms .......................................................................................................... 1 1.1.2 The marine carbohydrates ulvan and xylan ........................................................... 2 1.1.3 Marine polysaccharide utilization ........................................................................... 4 1.2 Carbohydrate-active enzymes -
The Metabolism of Tay-Sachs Ganglioside: Catabolic Studies with Lysosomal Enzymes from Normal and Tay-Sachs Brain Tissue
The Metabolism of Tay-Sachs Ganglioside: Catabolic Studies with Lysosomal Enzymes from Normal and Tay-Sachs Brain Tissue JOHN F. TALLMAN, WILLIAM G. JOHNSON, and ROSCOE 0. BRADY From the Developmental and Metabolic Neurology Branch, National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland 20014, and the Department of Biochemistry, Georgetown University School of Medicine, Washington, D. C. 20007 A B S T R A C T The catabolism of Tay-Sachs ganglioside, date fronm the 19th century and over 599 cases have been N-acetylgalactosaminyl- (N-acetylneuraminosyl) -galac- reported (1). Onset of the disease is in the first 6 months tosylglucosylceramide, has been studied in lysosomal of life and is characterized by apathy, hyperacusis, motor preparations from normal human brain and brain ob- weakness, and appearance of a macular cherry-red spot tained at biopsy from Tay-Sachs patients. Utilizing Tay- in the retina. Seizures and progressive mental deteriora- Sachs ganglioside labeled with '4C in the N-acetylgalac- tion follow with blindness, deafness, and spasticity, lead- tosaminyl portion or 3H in the N-acetylneuraminosyl ing to a state of decerebrate rigidity. These infants usu- portion, the catabolism of Tay-Sachs ganglioside may be ally die by 3 yr of age (2). initiated by either the removal of the molecule of A change in the chemical composition of the brain of N-acetylgalactosamine or N-acetylneuraminic acid. The such patients was first detected by Klenk who showed activity of the N-acetylgalactosamine-cleaving enzyme that there was an increase in the ganglioside content (hexosaminidase) is drastically diminished in such compared with normal human brain tissue (3). -
Bimodal Dynamics of Granular Organelles in Primary Renin- Expressing Cells Revealed Using TIRF Microscopy
Heriot-Watt University Research Gateway Bimodal dynamics of granular organelles in primary renin- expressing cells revealed using TIRF microscopy Citation for published version: Buckley, C, Dun, AR, Peter, A, Bellamy, C, Gross, KW, Duncan, RR & Mullins, JJ 2017, 'Bimodal dynamics of granular organelles in primary renin-expressing cells revealed using TIRF microscopy', AJP - Renal Physiology , vol. 312, no. 1, pp. F200-F209. https://doi.org/10.1152/ajprenal.00384.2016 Digital Object Identifier (DOI): 10.1152/ajprenal.00384.2016 Link: Link to publication record in Heriot-Watt Research Portal Document Version: Peer reviewed version Published In: AJP - Renal Physiology General rights Copyright for the publications made accessible via Heriot-Watt Research Portal is retained by the author(s) and / or other copyright owners and it is a condition of accessing these publications that users recognise and abide by the legal requirements associated with these rights. Take down policy Heriot-Watt University has made every reasonable effort to ensure that the content in Heriot-Watt Research Portal complies with UK legislation. If you believe that the public display of this file breaches copyright please contact [email protected] providing details, and we will remove access to the work immediately and investigate your claim. Download date: 27. Sep. 2021 1 Title Page 2 Title: 3 Bimodal dynamics of granular organelles in primary renin-expressing cells revealed using TIRF 4 microscopy 5 6 Running Head: 7 Imaging granule organelle dynamics in renin cells 8 9 Corresponding Author: 10 Charlotte Buckley 1 11 [email protected] 12 13 Other Authors: 14 Alison Dun 2 15 Audrey Peter 1 16 Christopher Bellamy3 17 Kenneth W. -
GM2 Gangliosidoses: Clinical Features, Pathophysiological Aspects, and Current Therapies
International Journal of Molecular Sciences Review GM2 Gangliosidoses: Clinical Features, Pathophysiological Aspects, and Current Therapies Andrés Felipe Leal 1 , Eliana Benincore-Flórez 1, Daniela Solano-Galarza 1, Rafael Guillermo Garzón Jaramillo 1 , Olga Yaneth Echeverri-Peña 1, Diego A. Suarez 1,2, Carlos Javier Alméciga-Díaz 1,* and Angela Johana Espejo-Mojica 1,* 1 Institute for the Study of Inborn Errors of Metabolism, Faculty of Science, Pontificia Universidad Javeriana, Bogotá 110231, Colombia; [email protected] (A.F.L.); [email protected] (E.B.-F.); [email protected] (D.S.-G.); [email protected] (R.G.G.J.); [email protected] (O.Y.E.-P.); [email protected] (D.A.S.) 2 Faculty of Medicine, Universidad Nacional de Colombia, Bogotá 110231, Colombia * Correspondence: [email protected] (C.J.A.-D.); [email protected] (A.J.E.-M.); Tel.: +57-1-3208320 (ext. 4140) (C.J.A.-D.); +57-1-3208320 (ext. 4099) (A.J.E.-M.) Received: 6 July 2020; Accepted: 7 August 2020; Published: 27 August 2020 Abstract: GM2 gangliosidoses are a group of pathologies characterized by GM2 ganglioside accumulation into the lysosome due to mutations on the genes encoding for the β-hexosaminidases subunits or the GM2 activator protein. Three GM2 gangliosidoses have been described: Tay–Sachs disease, Sandhoff disease, and the AB variant. Central nervous system dysfunction is the main characteristic of GM2 gangliosidoses patients that include neurodevelopment alterations, neuroinflammation, and neuronal apoptosis. Currently, there is not approved therapy for GM2 gangliosidoses, but different therapeutic strategies have been studied including hematopoietic stem cell transplantation, enzyme replacement therapy, substrate reduction therapy, pharmacological chaperones, and gene therapy. -
Genetics and Epigenetics in Parkinson´S Disease
Genetics and epigenetics in Parkinson´s disease. Luis Navarro Sánchez. Thesis supervisor: Jordi Pérez Tur. Unitat de Genètica Molecular, Institut de Biomedicina de València, CSIC. Doctoral studies: Biotechnology. Facultat de Ciències Biològiques, Universitat de València. Tras preguntas y más preguntas y miedo y nervios y ansiedad y más ansiedad y bloqueos y periodos depresivos y tristeza y mucha, pero mucha, mierda… acabas. Tarde y reventado, pero acabas. Y lo único que quieres es pasar página y que esto no te pase más nunca. ¿Y? Pues que te surgen más preguntas y más miedo y más nervios…. Y te das cuenta de que vives en un puto bucle científico que se está comiendo tu vida. ¿Y? No lo sé, estoy en ello. Terapia y medicación, supongo. Aunque la autodestrucción y los realities son muy apetecibles. En primer lugar, las formalidades. Gracias Jordi por dejarme que trabajara en la UGM. A estas alturas, creo que los dos nos habremos planteado muchas veces si fue una buena decisión, pero ya da igual. A continuación, el momento pasteleo, aunque si no sé dar un abrazo, no penséis que esto me va a salir fluido. Y menos aún, estando sobrio. Y que no se me enfade nadie si no he puesto su nombre o si no he escrito un parrafazo lacrimógeno y azucarado exaltando la belleza de nuestra relación. Si dejo que leas esto, significa que también eres importante para mí. Lo mejor de todo este tiempo sois, de calle, vosotros y vosotras: Bea, Carolina, Eva, Fer, LauraA, LauraG, María, Marta, Pili, Silvia, Vicente/Andreu y VicenteH.