COMP Fragment

Full Name

Cartilage oligomeric matrix protein Fragment

Keywords

MED; EDM1; EPD1; TSP5; PSACH; THBS5; cartilage oligomeric matrix protein; cartilage oligomeric matrix protein (pseudoachondroplasia, epiphyseal dysplasia 1, multiple); pseudoachondroplasia (epiphyseal dysplasia 1, multiple); thrombospondin-5

Background

The protein is a noncollagenous extracellular matrix (ECM) protein. It consists of five identical glycoprotein subunits, each with EGF-like and calcium-binding (thrombospondin-like) domains. Oligomerization results from formation of a five-stranded coiled coil and disulfides. Binding to other ECM proteins such as collagen appears to depend on divalent cations. Contraction or expansion of a 5 aa aspartate repeat and other mutations can cause pseudochondroplasia (PSACH) and multiple epiphyseal dysplasia (MED).