Article
Model systems for studying skeletal dysplasias caused by TSP-5/COMP mutations.
Cellular and molecular life sciences : CMLS - 1 Mar 2008
Posey K L, Yang Y, Veerisetty A C, Sharan S K, Hecht J T
Abstract excerpt
Cartilage oligomeric matrix protein, also known as thrombospondin-5 (TSP-5), is an extracellular matrix protein found primarily in cartilage and musculoskeletal tissues. TSP-5 is of interest because mutations in the gene cause two skeletal dysplasias, pseudoachondroplasia (PSACH) and multiple epiphyseal dysplasia (MED/EDM1). Both PSACH and EDM1 have a characteristic chondrocyte phenotype distinguished by giant...
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