Open AccessChondrocytes 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(s) -
Xu Maojia,
Stattin Eva-Lena,
Shaw Georgina,
Heinegård Dick,
Sullivan Gareth,
Wilmut Ian,
Colman Alan,
Önnerfjord Patrik,
Khabut Areej,
Aspberg Anders,
Dockery Peter,
Hardingham Timothy,
Murphy Mary,
Barry Frank
Publication year - 2016
Publication title -
stem cells translational medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.781
H-Index - 71
eISSN - 2157-6580
pISSN - 2157-6564
DOI - 10.5966/sctm.2015-0384
Subject(s) - endoplasmic reticulum , mesenchymal stem cell , microbiology and biotechnology , osteochondritis dissecans , matrix (chemical analysis) , extracellular matrix , chondrocyte , anatomy , chemistry , medicine , biology , cartilage , chromatography
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.