Recombinant Human Platelet glycoprotein Ib beta chain (GP1BB), Truncated

Uniprot Id

P13224

Target Species

Human

Target Name

GP1BB

Target Full Name

Platelet glycoprotein Ib beta chain

Target 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.

Target Involvement

Bernard-Soulier syndrome (BSS)

Target Subcellular Location

Membrane; Single-pass type I membrane protein.

Target Tissue Specificity

Expressed in heart and brain.

Target Research Area

Others

Target Synonyms

GP1BB; Platelet glycoprotein Ib beta chain; GP-Ib beta; GPIb-beta; GPIbB; Antigen CD42b-beta; CD antigen CD42c

Target Background

Platelet glycoprotein Ib (GPIb) is a heterodimeric transmembrane protein consisting of a disulfide-linked 140 kD alpha chain and 22 kD beta chain. It is part of the GPIb-V-IX system that constitutes the receptor for von Willebrand factor (VWF), and mediates platelet adhesion in the arterial circulation. GPIb alpha chain provides the VWF binding site, and GPIb beta contributes to surface expression of the receptor and participates in transmembrane signaling through phosphorylation of its intracellular domain. Mutations in the GPIb beta subunit have been associated with Bernard-Soulier syndrome, velocardiofacial syndrome and giant platelet disorder. The 206 amino acid precursor of GPIb beta is synthesized from a 1.0 kb mRNA expressed in plateletes and megakaryocytes. A 411 amino acid protein arising from a longer, unspliced transcript in endothelial cells has been described; however, the authenticity of this product has been questioned. Yet another less abundant GPIb beta mRNA species of 3.5 kb, expressed in nonhematopoietic tissues such as endothelium, brain and heart, was shown to result from inefficient usage of a non-consensus polyA signal in the neighboring upstream gene (SEPT5, septin 5). In the absence of polyadenylation from its own imperfect site, the SEPT5 gene produces read-through transcripts that use the consensus polyA signal of this gene.