The
protein encoded by this gene 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
disulfide bonds. Binding to other ECM proteins such as collagen appears to depend on divalent cations. Mutations can cause the osteochondrodysplasias
pseudoachondroplasia (PSACH) and
multiple epiphyseal dysplasia (MED). It is present in high quantities in
fibrotic scars and
systemic sclerosis, and it appears to have a role in vascular wall remodeling. == References ==