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Protein Page:
COMP (human)

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. Note: This description may include information from UniProtKB.
Protein type: Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 19p13.1
Cellular Component: extracellular matrix; extracellular region; extracellular space
Molecular Function: calcium ion binding; collagen binding; extracellular matrix structural constituent; heparan sulfate proteoglycan binding; heparin binding; protease binding; protein binding
Biological Process: extracellular matrix organization and biogenesis; limb development; negative regulation of apoptosis; organ morphogenesis; skeletal development
Disease: Epiphyseal Dysplasia, Multiple, 1; Pseudoachondroplasia
Reference #:  P49747 (UniProtKB)
Alt. Names/Synonyms: Cartilage oligomeric matrix protein; cartilage oligomeric matrix protein (pseudoachondroplasia, epiphyseal dysplasia 1, multiple); cartilage oligomeric matrix protein(pseudoachondroplasia, epiphyseal dysplasia 1, multiple); COMP; EDM1; EPD1; MED; MGC131819; MGC149768; PSACH; pseudoachondroplasia (epiphyseal dysplasia 1, multiple); THBS5; Thrombospondin-5; TSP5
Gene Symbols: COMP
Molecular weight: 82,860 Da
Basal Isoelectric point: 4.36  Predict pI for various phosphorylation states
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