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  • Xu, MaojiaNatl Univ Ireland Galway, Sch Med, Regenerat Med Inst, Galway, Ireland.,National University of Ireland Galway (author)

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

  • Article/chapterEnglish2016

Publisher, publication year, extent ...

  • 2016-07-07
  • Oxford University Press (OUP),2016
  • printrdacarrier

Numbers

  • LIBRIS-ID:oai:DiVA.org:uu-307869
  • https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-307869URI
  • https://doi.org/10.5966/sctm.2015-0384DOI
  • https://lup.lub.lu.se/record/233f23a3-c209-4c45-bf8d-cee19e9f60e4URI

Supplementary language notes

  • Language:English
  • Summary in:English

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  • Subject category:ref swepub-contenttype
  • Subject category:art swepub-publicationtype

Notes

  • Familial osteochondritis dissecans (FOCD) is an inherited skeletal defect characterized by the development of large cartilage lesions in multiple joints, short stature, and early onset of severe osteoarthritis. It is associated with a heterozygous mutation in the ACAN gene, resulting in a Val-Met replacement in the C-type lectin domain of aggrecan. To understand the cellular pathogenesis of this condition, we studied the chondrogenic differentiation of patient bone marrow mesenchymal stromal cells (BM-MSCs). We also looked at cartilage derived from induced pluripotent stem cells (iPSCs) generated from patient fibroblasts. Our results revealed several characteristics of the differentiated chondrocytes that help to explain the disease phenotype and susceptibility to cartilage injury. First, patient chondrogenic pellets had poor structural integrity but were rich in glycosaminoglycan. Second, it was evident that large amounts of aggrecan accumulated within the endoplasmic reticulum of chondrocytes differentiated from both BM-MSCs and iPSCs. In turn, there was a marked absence of aggrecan in the extracellular matrix. Third, it was evident that matrix synthesis and assembly were globally dysregulated. These results highlight some of the abnormal aspects of chondrogenesis in these patient cells and help to explain the underlying cellular pathology. The results suggest that FOCD is a chondrocyte aggrecanosis with associated matrix dysregulation. The work provides a new in vitro model of osteoarthritis and cartilage degeneration based on the use of iPSCs and highlights how insights into disease phenotype and pathogenesis can be uncovered by studying differentiation of patient stem cells.

Subject headings and genre

Added entries (persons, corporate bodies, meetings, titles ...)

  • Stattin, Eva-LenaUppsala University,Uppsala universitet,Medicinsk genetik och genomik,Science for Life Laboratory, SciLifeLab(Swepub:uu)evast375 (author)
  • Shaw, GeorginaNatl Univ Ireland Galway, Sch Med, Regenerat Med Inst, Galway, Ireland.,National University of Ireland Galway (author)
  • Heinegård, DickLund University,Lunds universitet,Reumatologi och molekylär skelettbiologi,Sektion III,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Rheumatology,Section III,Department of Clinical Sciences, Lund,Faculty of Medicine(Swepub:lu)medk-dhe (author)
  • Sullivan, GarethNorwegian Ctr Stem Cell Res, Oslo, Norway.,Norwegian Center for Stem Cell Research (author)
  • Wilmut, IanUniv Edinburgh, MRC, Ctr Regenerat Med, Edinburgh, Midlothian, Scotland.,University of Edinburgh (author)
  • Colman, AlanA STAR Inst Med Biol, Singapore, Singapore.,A*STAR Institute of Medical Biology (IMB) (author)
  • Önnerfjord, PatrikLund University,Lunds universitet,Reumatologi och molekylär skelettbiologi,Sektion III,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Rheumatology,Section III,Department of Clinical Sciences, Lund,Faculty of Medicine(Swepub:lu)akem-pon (author)
  • Khabut, AreejLund University,Lunds universitet,Reumatologi och molekylär skelettbiologi,Sektion III,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Rheumatology,Section III,Department of Clinical Sciences, Lund,Faculty of Medicine(Swepub:lu)med-akt (author)
  • Aspberg, AndersLund University,Lunds universitet,Reumatologi och molekylär skelettbiologi,Sektion III,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Rheumatology,Section III,Department of Clinical Sciences, Lund,Faculty of Medicine(Swepub:lu)medk-aas (author)
  • Dockery, PeterNatl Univ Ireland Galway, Sch Med, Dept Anat, Galway, Ireland.,National University of Ireland Galway (author)
  • Hardingham, TimothyUniv Manchester, Fac Life Sci, Wellcome Trust Ctr Cell Matrix Res, Manchester, Lancs, England.,University of Manchester (author)
  • Murphy, MaryNatl Univ Ireland Galway, Sch Med, Regenerat Med Inst, Galway, Ireland.,National University of Ireland Galway (author)
  • Barry, FrankNatl Univ Ireland Galway, Sch Med, Regenerat Med Inst, Galway, Ireland.,National University of Ireland Galway (author)
  • Natl Univ Ireland Galway, Sch Med, Regenerat Med Inst, Galway, Ireland.National University of Ireland Galway (creator_code:org_t)

Related titles

  • In:Stem Cells Translational Medicine: Oxford University Press (OUP)5:9, s. 1171-11812157-65642157-6580

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