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Publication : Cartilage oligomeric matrix protein-deficient mice have normal skeletal development.

First Author  Svensson L Year  2002
Journal  Mol Cell Biol Volume  22
Issue  12 Pages  4366-71
PubMed ID  12024046 Mgi Jnum  J:76781
Mgi Id  MGI:2180269 Doi  10.1128/MCB.22.12.4366-4371.2002
Citation  Svensson L, et al. (2002) Cartilage oligomeric matrix protein-deficient mice have normal skeletal development. Mol Cell Biol 22(12):4366-71
abstractText  Cartilage oligomeric matrix protein (COMP) belongs to the thrombospondin family and is a homopentamer primarily expressed in cartilage. Mutations in the COMP gene result in the autosomal dominant chondrodysplasias pseudoachondroplasia (PSACH) and some types of multiple epiphyseal dysplasia (MED), which are characterized by mild to severe short-limb dwarfism and early-onset osteoarthritis. We have generated COMP-null mice to study the role of COMP in vivo. These mice show no anatomical, histological, or ultrastructural abnormalities and show none of the clinical signs of PSACH or MED. Northern blot analysis and immunohistochemical analysis of cartilage indicate that the lack of COMP is not compensated for by any other member of the thrombospondin family. The results also show that the phenotype in PSACH/MED cartilage disorders is not caused by the reduced amount of COMP.
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