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FLASH GENE
Symbol COMP contributors: mct/npt/pgu - updated : 22-06-2015
HGNC name cartilage oligomeric matrix protein
HGNC id 2227
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • N-terminal aliphatic residues dictate the structure, stability, assembly, and small molecule binding of the coiled-coil region
  • a signal peptide
  • thrombospondin type 2 and 3 repeats, lacking the procollagen homology domain and seven type III repeats (TSP) of THBS1
  • four coiled-coil type II (EGF-like)
  • type III calmodulin-like (Ca++ binding) repeats, including a very short triplet repeat, encoding five Asp (GAC5) slightly expanded in some patients
    • conjugated GlycoP
    mono polymer homomer , pentamer
    HOMOLOGY
    interspecies homolog to murine Comp
    Homologene
    FAMILY
  • thrombospondin family
  • noncollagenous extracellular matrix family
    • CATEGORY adhesion , structural protein
    SUBCELLULAR LOCALIZATION extracellular
        intracellular
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    text
  • in the extracellular matrix (ECM) of developing and mature cartilage, within and around the tendon, in ligament, synovium and the vitreous of the eye
  • COMP and ECM1 colocalize in the growth plates
    • basic FUNCTION
  • structural protein with modified conformation when calcium is removed
  • playing with MATN3 an important role in matrix assembly
  • may function to stabilize the articular cartilage extracellular matrix by specific cation-dependent interactions with matrix components, including collagen types II and IX, fibronectin, aggrecan, and matrilin-1, -3, and -4
  • may act as the co-factor of the GRN cell surface receptor and may present GRN to its receptor, followed by the activations of GRN-mediated signal transduction and gene regulation pathways
  • playing a role in catalyzing the assembly of collagen, promoting formation of well defined fibrils
  • promotes cell attachment via two independent mechanisms involving cell surface CD47 and alphaVbeta3 integrin (and consequence of cell attachment to COMP is the specific induction of fascin-stabilized actin microspikes) (Rock 2010)
  • pivotal for maintaining the homeostasis of vascular smooth muscle cells (VSMCs)
  • potential novel inhibitor of vascular calcificationand the imbalance between the effects of COMP and BMP2 may provide new insights into the pathophysiology of vascular calcification
  • functions as an adapter protein in human skin, similar to its function in cartilage ECM, by organizing collagen I fibrils into a suprastructure, mainly in the vicinity of anchoring plaques that stabilize the cohesion between the upper dermis and the basement membrane zone
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS development
    text human limb development
    PATHWAY
    metabolism
    signaling
    a component
  • pentameric approximately 524 kDa multidomain extracellular matrix protein (R
    • INTERACTION
    DNA
    RNA
    small molecule metal binding,
  • Ca2+
    • protein
  • interacting with GRN (required for GRN-mediated chondrocyte proliferation, since chondrocyte proliferation induced by GRN is dramatically inhibited by an anti-COMP antibody)
  • ADAMTS12 is a new COMP-interacting and -degrading enzyme and thus may play an important role in the COMP degradation in the initiation and progression of arthritis
  • binding to collagens I and II is enhanced in the presence of Zn2+
  • ADAMTS7 facilitated intimal hyperplasia through degradation of inhibitory matrix protein COMP
  • ECM1 novel COMP-associated partner (EGF domain of COMP and the C-terminus of ECM1 mediate the interaction between them)
  • bound directly to BMP2 through the C-terminus, inhibited BMP2 receptor binding, and blocked BMP2 osteogenic signaling
  • COMP binds to COL12A1 and COL14A1 via their C-terminal collagenous domains, and three proteins codistribute in a characteristic narrow zone in the superficial papillary dermis of healthy human skin
  • ADAMTS7 facilitated vascular smooth muscle cell (VSMC) migration by degrading the extracellular matrix protein COMP and thereby promoted neointima formation following vascular mechanical injury
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) EDM1 , PSACH
    Susceptibility putative susceptibility gene for rheumatoid arthritis
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS