Product Overview
Description
CLPP-00150476 is recombinant human COMP protein
Applications
SDS-PAGE, Western Blot
Protein Length
Full length protein
Nature
Recombinant Protein
Sequence
HVDYKDDDDKPAGQGQSPLGSDLGPQMLRELQETNAALQDVRE
LLRQQVREITFLKNTVMECDACGMQQSVRTGLPSVRPLLHCAPGFCFPGV
ACIQTESGARCGPCPAGFTGNGSHCTDVNECNAHPCFPRVRCINTSPGFR
CEACPPGYSGPTHQGVGLAFAKANKQVCTDINECETGQHNCVPNSVCINTRGSFQCGPCQPGFVGDQASGCQRRAQRFCPDGSPSECHEHADCVLERDGS
RSCVCAVGWAGNGILCGRDTDLDGFPDEKLRCPERQCRKDNCVTVPNSGQ
EDVDRDGIGDACDPDADGDGVPNEKDNCPLVRNPDQRNTDEDKWGDACDN
CRSQKNDDQKDTDQDGRGDACDDDIDGDRIRNQADNCPRVPNSDQKDSDG
DGIGDACDNCPQKSNPDQADVDHDFVGDACDSDQDQDGDGHQDSRDNCPT
VPNSAQEDSDHDGQGDACDDDDDNDGVPDSRDNCRLVPNPGQEDADRDGV
GDVCQDDFDADKVVDKIDVCPENAEVTLTDFRAFQTVVLDPEGDAQIDPN
WVVLNQGREIVQTMNSDPGLAVGYTAFNGVDFEGTFHVNTVTDDDYAGFI
FGYQDSSSFYVVMWKQMEQTYWQANPFRAVAEPGIQLKAVKSSTGPGEQL
RNALWHTGDTESQVRLLWKDPRNVGWKDKKSYRWFLQHRPQVGYIRVRFY
EGPELVADSNVVLDTTMRGGRLGVFCFSQENIIWANLRYRCNDTIPEDYE
THQLRQA
Sequence Similarities
Belongs to the thrombospondin family. Contains 4 EGF-like domains. Contains 1 TSP C-terminal (TSPC) domain. Contains 8 TSP type-3 repeats.
Predicted Molecular Weight
82 kDa including tags
Tags
DDDDK tag N-Terminus
Target Information
Alternative Names
Cartilage oligomeric matrix protein; cartilage oligomeric matrix protein (pseudoachondroplasia, epiphyseal dysplasia 1, multiple); Cartilage oligomeric matrix protein precursor; COMP; COMP_HUMAN; EDM 1; EDM1; EPD 1; EPD1; Epiphyseal dysplasia 1; Epiphyseal dysplasia 1 multiple; Epiphyseal dysplasia multiple 1; MED; MGC13181; MGC149768; PSACH; Pseudoachondroplasia; pseudoachondroplasia (epiphyseal dysplasia 1, multiple); THBS 5; THBS5; Thrombospondin 5; Thrombospondin-5; Thrombospondin5; TSP5
Protein Function
Plays 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 (By similarity).
Tissue Specificity
Abundantly expressed in the chondrocyte extracellular matrix, and is also found in bone, tendon, ligament and synovium and blood vessels. Increased amounts are produced during late stages of osteoarthritis in the area adjacent to the main defect.
Involvement in Disease
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.
Shipping & Handling
Constituents
0.242% Tris, 0.29% Sodium chloride.
Shipping
Shipped on dry ice.
Storage
Store at -20 °C for long term.
