Description
Product Characteristics: The antibody is directed against the CD42b antigen, platelet glycoprotein gp1bA, that serves as a receptor for Von Willebrand factor (vWF) and as a high affinity thrombin receptor. AK2 immunblots a band of 130kDa on SDS gels of platelet lysates and gives surface immunofluorescence with fixed or unfixed platelets. The antigen is absent in very low levels on platelets with the Bernard-Soulier syndrome.
Target Information: 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. Several polymorphisms and mutations have been described in this gene, some of which are the cause of Bernard-Soulier syndromes and platelet-type von Willebrand disease. [provided by RefSeq, Mar 2010]