蛋白名称
Platelet glycoprotein Ib alpha chain
免疫原
The antiserum was produced against synthesized peptide derived from the Internal region of human GP1BA. AA range:271-320
特异性
CD42b Polyclonal Antibody detects endogenous levels of CD42b protein.
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
来源
Polyclonal, Rabbit,IgG
稀释比例
WB: 1/500 - 1/2000. IHC-p: 1/100-1/300. ELISA: 1/20000.. IF 1:50-200
纯化工艺
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
其他名称
GP1BA; Platelet glycoprotein Ib alpha chain; GP-Ib alpha; GPIb-alpha; GPIbA; Glycoprotein Ibalpha; Antigen CD42b-alpha; CD42b
背景
Glycoprotein Ib (GP Ib) is a platelet surface membrane glycoprotein composed of a heterodimer, an alpha chain and a beta chain, that is linked by disulfide bonds. The Gp Ib functions as a receptor for von Willebrand factor (VWF). The complete receptor complex includes noncovalent association of the alpha and beta subunits with platelet glycoprotein IX and platelet glycoprotein V. The binding of the GP Ib-IX-V complex to VWF facilitates initial platelet adhesion to vascular subendothelium after vascular injury, and also initiates signaling events within the platelet that lead to enhanced platelet activation, thrombosis, and hemostasis. This gene encodes the alpha subunit. Mutations in this gene result in Bernard-Soulier syndromes and platelet-type von Willebrand disease. The coding region of this gene is known to contain a polymophic variable number tandem repeat (VNTR) domain that is
功能
disease:Defects in GP1BA 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.,disease:Defects in GP1BA are a cause of von Willebrand disease (vWD) [MIM:177820]; also known as platelet-type von Willebrand disease or pseudo-von Willebrand disease (pseudo-vWD). This autosomal dominant bleeding disorder is caused by an increased affinity of GP-Ib for soluble vWF resulting in impaired hemostatic function due to the removal of vWF from the circulation.,disease:Defects in GP1BA are the cause of benign mediterranean macrothrombocytopenia [MIM:153670]; also known as autosomal dominant benign Bernard-Soulier syndrome. Benign mediterranean macrothrombocytopenia is characterized by mild or no clinical symptoms, normal platelet function, and normal megakaryocyte count.
