Home
>
Product
>
Antibody
>
Rabbit Anti-Glycoprotein VI/FITC Conjugated antibody
background:
This gene encodes a platelet membrane glycoprotein of the immunoglobulin superfamily. The encoded protein is a receptor for collagen and plays a critical role in collagen-induced platelet aggregation and thrombus formation. The encoded protein forms a complex with the Fc receptor gamma-chain that initiates the platelet activation signaling cascade upon collagen binding. Mutations in this gene are a cause of platelet-type bleeding disorder-11 (BDPLT11). Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene. [provided by RefSeq, Dec 2011].
Function:
Collagen receptor involved in collagen-induced platelet adhesion and activation. Plays a key role in platelet procoagulant activity and subsequent thrombin and fibrin formation. This procoagulant function may contribute to arterial and venous thrombus formation. The signaling pathway involves the FcR gamma-chain, the Src kinases (likely Fyn/Lyn), the adapter protein LAT and leads to the activation of phospholipase C gamma2.
Subunit:
Associated with Fc receptor gamma chain. The GPVI-FcRgamma complex is associated with the Src kinase family Fyn and Lyn.
Subcellular Location:
Isoform 1: Cell membrane; Single-pass membrane protein.
Isoform 2: Cell membrane; Single-pass membrane protein.
Tissue Specificity:
Megakaryocytes and platelets.
Post-translational modifications:
N-linked glycosylation at Asn-92 is not required for the cell surface expression, but contributes to maximal adhesion to type I collagen, collagen-related peptide (CRP), and, to a lesser extent, to the snake venom SLCtype lectin convulxin (CVX).
DISEASE:
Defects in GP6 are the cause of bleeding disorder platelet-type 11 (BDPLT11) [MIM:614201]. BDPLT11 is a mild to moderate bleeding disorder caused by defective platelet activation and aggregation in response to collagen.
Similarity:
Contains 2 Ig-like C2-type (immunoglobulin-like) domains.
Database links:
Entrez Gene: 51206 Human
SwissProt: Q9HCN6 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
|
|