International Journal of Molecular Sciences Extracellular Matrix in Development and Disease Edited by Julia Thom Oxford Printed Edition of the Special Issue Published in International Journal of Molecular Sciences www.mdpi.com/journal/ijms Extracellular Matrix in Development and Disease Extracellular Matrix in Development and Disease Special Issue Editor Julia Thom Oxford MDPI • Basel • Beijing • Wuhan • Barcelona • Belgrade Special Issue Editor Julia Thom Oxford Boise State University USA Editorial Office MDPI St. Alban-Anlage 66 4052 Basel, Switzerland This is a reprint of articles from the Special Issue published online in the open access journal International Journal of Molecular Sciences (ISSN 1422-0067) from 2017 to 2019 (available at: https: //www.mdpi.com/journal/ijms/special issues/extracellular matrix) For citation purposes, cite each article independently as indicated on the article page online and as indicated below: LastName, A.A.; LastName, B.B.; LastName, C.C. Article Title. Journal Name Year, Article Number, Page Range. ISBN 978-3-03897-570-0 (Pbk) ISBN 978-3-03897-571-7 (PDF) Cover image courtesy of Roxanne Stone and Stephanie Frahs; scanning electron micrograph acquired by Makenna Hardy, Boise State University. The cover image depicts a decellularized cartilage ECM scaffold seeded with chondrocytes. c 2019 by the authors. Articles in this book are Open Access and distributed under the Creative Commons Attribution (CC BY) license, which allows users to download, copy and build upon published articles, as long as the author and publisher are properly credited, which ensures maximum dissemination and a wider impact of our publications. The book as a whole is distributed by MDPI under the terms and conditions of the Creative Commons license CC BY-NC-ND. Contents About the Special Issue Editor ...................................... vii Julia Thom Oxford, Jonathon C. Reeck and Makenna J. Hardy Extracellular Matrix in Development and Disease Reprinted from: Int. J. Mol. Sci. 2019, 20, 205, doi:10.3390/ijms20010205 ............... 1 Lavinia Arseni, Anita Lombardi and Donata Orioli From Structure to Phenotype: Impact of Collagen Alterations on Human Health Reprinted from: Int. J. Mol. Sci. 2018, 19, 1407, doi:10.3390/ijms19051407 .............. 6 Salin A. Chakkalakal, Juliane Heilig, Ulrich Baumann, Mats Paulsson and Frank Zaucke Impact of Arginine to Cysteine Mutations in Collagen II on Protein Secretion and Cell Survival Reprinted from: Int. J. Mol. Sci. 2018, 19, 541, doi:10.3390/ijms19020541 ............... 42 Sara Stridh, Fredrik Palm, Tomoko Takahashi, Mayumi Ikegami-Kawai, Malou Friederich-Persson and Peter Hansell Hyaluronan Production by Renomedullary Interstitial Cells: Influence of Endothelin, Angiotensin II and Vasopressin Reprinted from: Int. J. Mol. Sci. 2017, 18, 2701, doi:10.3390/ijms18122701 .............. 59 Eva Nagyova The Biological Role of Hyaluronan-Rich Oocyte-Cumulus Extracellular Matrix in Female Reproduction Reprinted from: Int. J. Mol. Sci. 2018, 19, 283, doi:10.3390/ijms19010283 ............... 74 Yao Ye, Aurelia Vattai, Xi Zhang, Junyan Zhu, Christian J. Thaler, Sven Mahner, Udo Jeschke and Viktoria von Sch¨onfeldt Role of Plasminogen Activator Inhibitor Type 1 in Pathologies of Female Reproductive Diseases Reprinted from: Int. J. Mol. Sci. 2017, 18, 1651, doi:10.3390/ijms18081651 .............. 88 Cameron Walker, Elijah Mojares and Armando del R´ıo Hern´andez Role of Extracellular Matrix in Development and Cancer Progression Reprinted from: Int. J. Mol. Sci. 2018, 19, 3028, doi:10.3390/ijms19103028 ..............105 Shike Wang, Zhenyu Li and Ren Xu Human Cancer and Platelet Interaction, a Potential Therapeutic Target Reprinted from: Int. J. Mol. Sci. 2018, 19, 1246, doi:10.3390/ijms19041246 ..............136 Maria Del Ben, Diletta Overi, Licia Polimeni, Guido Carpino, Giancarlo Labbadia, Francesco Baratta, Daniele Pastori, Valeria Noce, Eugenio Gaudio, Francesco Angelico and Carmine Mancone Overexpression of the Vitronectin V10 Subunit in Patients with Nonalcoholic Steatohepatitis: Implications for Noninvasive Diagnosis of NASH Reprinted from: Int. J. Mol. Sci. 2018, 19, 603, doi:10.3390/ijms19020603 ...............151 Girdhari Rijal, Jing Wang, Ilhan Yu, David R. Gang, Roland K. Chen and Weimin Li Porcine Breast Extracellular Matrix Hydrogel for Spatial Tissue Culture Reprinted from: Int. J. Mol. Sci. 2018, 19, 2912, doi:10.3390/ijms19102912 ..............165 v Kena Song, Zirui Wang, Ruchuan Liu, Guo Chen and Liyu Liu Microfabrication-Based Three-Dimensional (3-D) Extracellular Matrix Microenvironments for Cancer and Other Diseases Reprinted from: Int. J. Mol. Sci. 2018, 19, 935, doi:10.3390/ijms19040935 ...............178 Scott P. Levick and Alexander Widiapradja Mast Cells: Key Contributors to Cardiac Fibrosis Reprinted from: Int. J. Mol. Sci. 2018, 19, 231, doi:10.3390/ijms19010231 ...............192 Dimitry A. Chistiakov, Alexandra A. Melnichenko, Veronika A. Myasoedova, Andrey V. Grechko and Alexander N. Orekhov Thrombospondins: A Role in Cardiovascular Disease Reprinted from: Int. J. Mol. Sci. 2017, 18, 1540, doi:10.3390/ijms18071540 ..............210 Ilonka Rohm, Katja Grun, ¨ Linda Marleen Muller, ¨ Daniel Kretzschmar, Michael Fritzenwanger, Atilla Yilmaz, Alexander Lauten, Christian Jung, P. Christian Schulze, Alexander Berndt and Marcus Franz Increased Serum Levels of Fetal Tenascin-C Variants in Patients with Pulmonary Hypertension: Novel Biomarkers Reflecting Vascular Remodeling and Right Ventricular Dysfunction? Reprinted from: Int. J. Mol. Sci. 2017, 18, 2371, doi:10.3390/ijms18112371 ..............239 Carolyn M. Dancevic, Yann Gibert, Joachim Berger, Adam D. Smith, Clifford Liongue, Nicole Stupka, Alister C. Ward and Daniel R. McCulloch The ADAMTS5 Metzincin Regulates Zebrafish Somite Differentiation Reprinted from: Int. J. Mol. Sci. 2018, 19, 766, doi:10.3390/ijms19030766 ...............250 Natasha McRae, Leonard Forgan, Bryony McNeill, Alex Addinsall, Daniel McCulloch, Chris Van der Poel and Nicole Stupka Glucocorticoids Improve Myogenic Differentiation In Vitro by Suppressing the Synthesis of Versican, a Transitional Matrix Protein Overexpressed in Dystrophic Skeletal Muscles Reprinted from: Int. J. Mol. Sci. 2017, 18, 2629, doi:10.3390/ijms18122629 ..............262 Mario Enrique Alvar`ez Fallas, Martina Piccoli, Chiara Franzin, Alberto Sgr`o, Arben Dedja, Luca Urbani, Enrica Bertin, Caterina Trevisan, Piergiorgio Gamba, Alan J. Burns, Paolo De Coppi and Michela Pozzobon Decellularized Diaphragmatic Muscle Drives a Constructive Angiogenic Response In Vivo Reprinted from: Int. J. Mol. Sci. 2018, 19, 1319, doi:10.3390/ijms19051319 ..............284 Anna Urciuolo and Paolo De Coppi Decellularized Tissue for Muscle Regeneration Reprinted from: Int. J. Mol. Sci. 2018, 19, 2392, doi:10.3390/ijms19082392 ..............303 Bryce LaFoya, Jordan A. Munroe, Alison Miyamoto, Michael A. Detweiler, Jacob J. Crow, Tana Gazdik and Allan R. Albig Beyond the Matrix: The Many Non-ECM Ligands for Integrins Reprinted from: Int. J. Mol. Sci. 2018, 19, 449, doi:10.3390/ijms19020449 ...............314 vi About the Special Issue Editor Julia Thom Oxford, Ph.D., is a Distinguished Professor and the Stueckle Endowed Chair in the Department of Biological Sciences. She received a Doctor of Philosophy degree in Biochemistry and Biophysics in 1986 from Washington State University and completed a postdoctoral fellowship at Shriners Hospital for Children focusing on the role of extracellular matrix in skeletal development. She began working at Boise State University in 2000 and has taught courses on the Extracellular Matrix and Developmental Biology since 2001. Her current research projects focus on rare diseases, molecular mechanisms of cellular mechanoreception in bone and cartilage, the regulation of cell signaling by Col11a1 during craniofacial development, the extracellular matrix as a key factor in cancer progression, the effects of simulated microgravity on articular cartilage, the induction of early stages of osteoarthritis, biomaterials, 3-D cell culture model systems, and alternative splicing of mRNA during embryonic development. She is the Program Director of the NIH Center of Biomedical Research Excellence in Matrix Biology. vii International Journal of Molecular Sciences Editorial Extracellular Matrix in Development and Disease Julia Thom Oxford 1,2,3,4,* , Jonathon C. Reeck 1,2 and Makenna J. Hardy 1,3 1 Center of Biomedical Research Excellence in Matrix Biology, Boise State University, Boise, ID 83725-1511, USA; [email protected] (J.C.R.); [email protected] (M.J.H.) 2 Biomolecular Research Center, Boise State University, Boise, ID 83725-1511, USA 3 Biomolecular Sciences Graduate Program, Boise State University, Boise, ID 83725, USA 4 Department of Biological Science, Boise State University, Boise, ID 83725-1515, USA * Correspondence: [email protected]; Tel.: +1-208-426-2395 Received: 19 December 2018; Accepted: 4 January 2019; Published: 8 January 2019 Keywords: extracellular matrix; hereditary diseases; reproduction; cancer; muscle; tissue engineering; integrins 1. Introduction The evolution of multicellular metazoan organisms was marked by the inclusion of an extracellular matrix (ECM), a multicomponent, proteinaceous network between cells that contributes to the spatial arrangement of cells and the resulting tissue organization. The development of an ECM that provides support in larger organisms may have represented an advantage in the face of selection