Abstract

Bone marrow-derived mesenchymal stromal cells (BM-MSCs) and induced pluripotent stem cells (iPSCs) from patients with familial osteochondritis dissecans (FOCD) were studied. Chondrogenic pellets had poor structural integrity but were rich in glycosaminoglycan. Large amounts of aggrecan accumulated within the endoplasmic reticulum of chondrocytes, with a marked absence of aggrecan in the extracellular matrix. Matrix synthesis and assembly were globally dysregulated. These results suggest that FOCD is a chondrocyte aggrecanosis with associated matrix dysregulation.

Details

Title
Chondrocytes Derived From Mesenchymal Stromal Cells and Induced Pluripotent Cells of Patients With Familial Osteochondritis Dissecans Exhibit an Endoplasmic Reticulum Stress Response and Defective Matrix Assembly
Author
Xu Maojia 1 ; Eva-Lena, Stattin 2 ; Shaw, Georgina 1 ; Heinegård Dick 3 ; Sullivan, Gareth 4 ; Wilmut, Ian 5 ; Colman, Alan 6 ; Önnerfjord Patrik 3 ; Khabut Areej 3 ; Aspberg Anders 3 ; Dockery, Peter 7 ; Hardingham, Timothy 8 ; Murphy, Mary 1 ; Barry, Frank 1 

 Regenerative Medicine Institute, National University of Ireland Galway, Galway, Ireland 
 Department of Immunology, Genetics, and Pathology, Science for Life Laboratory, Uppsala University, Uppsala, Sweden 
 Department of Clinical Sciences, Rheumatology, and Molecular Skeletal Biology, Lund University, Lund, Sweden 
 Norwegian Center for Stem Cell Research, Oslo, Norway 
 Medical Research Council Centre for Regenerative Medicine, University of Edinburgh, Edinburgh, United Kingdom 
 A*STAR Institute of Medical Biology, Singapore 
 Anatomy, School of Medicine, National University of Ireland Galway, Galway, Ireland 
 Wellcome Trust Centre for Cell-Matrix Research, Faculty of Life Sciences University of Manchester, Manchester, United Kingdom 
Pages
1171-1181
Section
Tissue-Specific Progenitor and Stem Cells
Publication year
2016
Publication date
Sep 2016
Publisher
Oxford University Press
ISSN
21576564
e-ISSN
21576580
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
2333897727
Copyright
© 2016. This work is published under https://creativecommons.org/licenses/by/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.