Anti-human GpVI clone S-D2 Antibody against glycoprotein VI (GPVI, GP6), a platelet membrane glycoprotein of the immunoglobulin superfamily and a receptor for collagen involved in collagen-induced platelet adhesion and activation. Ligand binding to GPVI initiates migration to lipid rafts and subsequent dimerization of GPVI, the formation of a signaling complex with the FcR gamma chain, and the recruitment of downstream signaling proteins, including Src family kinases Fyn and Lyn and the adapter protein LAT. Activation of this signaling pathway results in thrombus formation via the activation of phospholipase C gamma 2. Transduction of signals by GPVI is mediated in an immunoreceptor-based manner and involves its immune-receptor tyrosine-based activation motif (ITAM). In addition to collagen or collagen-related peptides, GPVI was reported to also bind other ligands, including fibrinogen and fibrin, resulting in the support of growth and stabilization of the thrombus. Different inherited and acquired disease-causing variants of GPVI are known, which include mutations in the gp6 gene causing the bleeding disorder platelet-type 11 (BDPLT11), a mild to moderate bleeding disorder characterized by defective platelet activation and aggregation in response to collagen. Also, platelets may be deficient of GPVI due to inherited or acquired loss of the protein, the latter through i.e. autoantibody-induced receptor shedding.
