Article
An inducible cartilage oligomeric matrix protein mouse model recapitulates human pseudoachondroplasia phenotype.
The American journal of pathology - 1 Oct 2009
Posey Karen L, Veerisetty Alka C, Liu Pieman, Wang Huiqiu R, Poindexter Brian J, Bick Roger, Alcorn Joseph L, Hecht Jacqueline T
Abstract excerpt
Cartilage oligomeric matrix protein (COMP) is a pentameric extracellular protein expressed in cartilage and other musculoskeletal tissues. Mutations in the COMP gene cause pseudoachondroplasia (PSACH), a severe dwarfing condition that has a growth plate chondrocyte pathology. PSACH is characterized by intracellular retention of COMP and other extracellular matrix (ECM) proteins, which form an ordered matrix...
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