功能： disease:Defects in GP1BB are a cause of Bernard-Soulier syndrome (BSS) [MIM:231200]; also known as giant platelet disease (GPD). BSS patients have unusually large platelets and have a clinical bleeding tendency.,function:Gp-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to von Willebrand factor, which is already bound to the subendothelium.,miscellaneous:Platelet activation apparently involves disruption of the macromolecular complex of GP-Ib with the platelet glycoprotein IX (GP-IX) and dissociation of GP-Ib from the actin-binding protein.,similarity:Contains 1 LRR (leucine-rich) repeat.,subunit:GP-Ib alpha and beta are disulfide linked. GP-IX is complexed with the GP-Ib heterodimer via a non covalent linkage.,tissue specificity:Expressed in heart and brain.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Membrane; Single-pass type I membrane protein.
功能： disease:Defects in GP1BB are a cause of Bernard-Soulier syndrome (BSS) [MIM:231200]; also known as giant platelet disease (GPD). BSS patients have unusually large platelets and have a clinical bleeding tendency.,function:Gp-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to von Willebrand factor, which is already bound to the subendothelium.,miscellaneous:Platelet activation apparently involves disruption of the macromolecular complex of GP-Ib with the platelet glycoprotein IX (GP-IX) and dissociation of GP-Ib from the actin-binding protein.,similarity:Contains 1 LRR (leucine-rich) repeat.,subunit:GP-Ib alpha and beta are disulfide linked. GP-IX is complexed with the GP-Ib heterodimer via a non covalent linkage.,tissue specificity:Expressed in heart and brain.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Membrane; Single-pass type I membrane protein.
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