Cartilage Oligomeric Matrix (COMP) ELISA Kit for Porcine

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 disulfides. Binding to other ECM proteins such as collagen appears to depend on divalent cations. Mutations can cause the osteochondrodysplasias pseudochondroplasia (PSACH) and multiple epiphyseal dysplasia (MED). [provided by RefSeq, Jul 2008]

COMP: May play a role in the structural integrity of cartilage via its interaction with other extracellular matrix proteins such as the collagens and fibronectin. Can mediate the interaction of chondrocytes with the cartilage extracellular matrix through interaction with cell surface integrin receptors. Could play a role in the pathogenesis of osteoarthritis. Potent suppressor of apoptosis in both primary chondrocytes and transformed cells. Suppresses apoptosis by blocking the activation of caspase-3 and by inducing the IAP family of survival proteins (BIRC3, BIRC2, BIRC5 and XIAP). Essential for maintaining a vascular smooth muscle cells (VSMCs) contractile/differentiated phenotype under physiological and pathological stimuli. Maintains this phenotype of VSMCs by interacting with ITGA7. Defects in COMP are the cause of multiple epiphyseal dysplasia type 1 (EDM1). EDM is a generalized skeletal dysplasia associated with significant morbidity. Joint pain, joint deformity, waddling gait, and short stature are the main clinical signs and symptoms. EDM is broadly categorized into the more severe Fairbank and the milder Ribbing types. Defects in COMP are the cause of pseudoachondroplasia (PSACH). PSAC is a dominantly inherited chondrodysplasia characterized by short stature and early-onset osteoarthrosis. PSACH is more severe than EDM1 and is recognized in early childhood. Belongs to the thrombospondin family.

Protein type: Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 19p13.1

Cellular Component: extracellular matrix; proteinaceous extracellular matrix; extracellular space; extracellular region

Molecular Function: collagen binding; heparan sulfate proteoglycan binding; heparin binding; protein binding; protease binding; extracellular matrix structural constituent; calcium ion binding

Biological Process: limb development; organ morphogenesis; extracellular matrix organization and biogenesis; apoptosis; cell adhesion; skeletal development; negative regulation of apoptosis

Disease: Epiphyseal Dysplasia, Multiple, 1; Pseudoachondroplasia