NP_000164.5
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
71,540 Da
NCBI Official Full Name
platelet glycoprotein Ib alpha chain
NCBI Official Synonym Full Names
glycoprotein Ib (platelet), alpha polypeptide
NCBI Official Synonym Symbols
BSS; GP1B; VWDP; CD42B; GPIbA; BDPLT1; BDPLT3; DBPLT3; CD42b-alpha [Similar Products]
NCBI Protein Information
platelet glycoprotein Ib alpha chain; GP-Ib alpha; antigen CD42b-alpha; platelet membrane glycoprotein 1b-alpha subunit
UniProt Protein Name
Platelet glycoprotein Ib alpha chain
UniProt Synonym Protein Names
Antigen CD42b-alpha; CD_antigen: CD42bGlycocalicin
UniProt Synonym Gene Names
UniProt Entry Name
GP1BA_HUMAN
NCBI Summary for CD42b
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 associated with susceptibility to nonarteritic anterior ischemic optic neuropathy. [provided by RefSeq, Oct 2013]
UniProt Comments for CD42b
GPIbA: GP-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to the A1 domain of vWF, which is already bound to the subendothelium. Genetic variations in GP1BA may be a cause of susceptibility to non-arteritic anterior ischemic optic neuropathy (NAION). NAION is an ocular disease due to ischemic injury to the optic nerve. It usually affects the optic disk and leads to visual loss and optic disk swelling of a pallid nature. Visual loss is usually sudden, or over a few days at most and is usually permanent, with some recovery possibly occurring within the first weeks or months. Patients with small disks having smaller or non-existent cups have an anatomical predisposition for non-arteritic anterior ischemic optic neuropathy. As an ischemic episode evolves, the swelling compromises circulation, with a spiral of ischemia resulting in further neuronal damage. Defects in GP1BA are a cause of Bernard-Soulier syndrome (BSS); also known as giant platelet disease (GPD). BSS patients have unusually large platelets and have a clinical bleeding tendency. Defects in GP1BA are the cause of benign mediterranean macrothrombocytopenia (BMM); also known as autosomal dominant benign Bernard-Soulier syndrome. BMM is characterized by mild or no clinical symptoms, normal platelet function, and normal megakaryocyte count. Defects in GP1BA are the cause of pseudo-von Willebrand disease (VWDP). A 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.
Protein type: Cell adhesion; Cell surface; Membrane protein, integral
Chromosomal Location of Human Ortholog: 17p13.2
Cellular Component: anchored to external side of plasma membrane; cell surface; membrane; integral to plasma membrane; plasma membrane
Molecular Function: protein binding; thrombin receptor activity
Biological Process: platelet activation; fibrinolysis; cell surface receptor linked signal transduction; regulation of blood coagulation; cell morphogenesis; blood coagulation; cell adhesion; blood coagulation, intrinsic pathway
Disease: Pseudo-von Willebrand Disease; Bernard-soulier Syndrome; Bernard-soulier Syndrome, Type A2, Autosomal Dominant; Nonarteritic Anterior Ischemic Optic Neuropathy, Susceptibility To
Research Articles on CD42b
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Products associated with anti-CD42b antibody
Pathways associated with anti-CD42b antibody
Diseases associated with anti-CD42b antibody
Organs/Tissues associated with anti-CD42b antibody
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