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Platelet glycoprotein Ib (GPIb) is a heterodimeric transmembrane protein consisting of a disulfide-linked 140 kD alpha chain and 22 kD beta chain. Zusätzlich bieten wir Ihnen GP1BB Antikörper (29) und GP1BB Proteine (11) und viele weitere Produktgruppen zu diesem Protein an.
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Rare variants in GP1BB are responsible for autosomal dominant macrothrombocytopenia.
miR (zeige MLXIP ELISA Kits)-10a and miR10b regulate the expression of human platelet GP1BA (zeige GP1BA ELISA Kits) and GP1bb for normal megakaryopoiesis.
Data show that localization of the GP Ib-IX complex to the lipid domain is mediated by GP Ibbeta and GP IX (zeige GP9 ELISA Kits) transmembrane domains.
Low levels of CD9 (zeige CD9 ELISA Kits) coincidental with a novel nonsense mutation in glycoprotein Ibbeta in a patient with Bernard-Soulier syndrome.
genetic association study in population in western India: Data suggest novel mutations in platelet glycoprotein Ib (GP1BA (zeige GP1BA ELISA Kits), GP1BB) and GP9 (zeige GP9 ELISA Kits) are associated with Bernard-Soulier syndrome in subjects studies; of 12 mutations identified, ten were novel.
a suspicion of 22q11.2 deletion is warranted in pediatric BSS (zeige GP1BA ELISA Kits) patients with a mutation in the GPIbbeta gene, even without remarkable symptoms.
Studies indicate that platelets from Bernard-Soulier syndrome (BSS (zeige GP1BA ELISA Kits)) are defective in glycoprotein (GP)Ib-IX-V, a platelet-specific adhesion-signaling complex, composed of GPIbalpha (zeige GP1BA ELISA Kits) disulfide linked to GPIbbeta, and noncovalently associated with GPIX (zeige GP9 ELISA Kits) and GPV (zeige GP5 ELISA Kits).
GPIbbeta missense mutations from Bernard-Soulier syndrome were examined for changes to GPIb-IX complex surface expression. Mutations A108P and P74R were found to maintain normal secretion/folding of GPIbbeta(E) but were unable to support GPIX (zeige GP9 ELISA Kits) surface expression
GPIIb/IIIa is the primary receptor set involved in platelet adhesion to adsorbed fibrinogen and serum albumin (zeige ALB ELISA Kits) irrespective of their degree of adsorption-induced unfolding, while the GPIb-IX-V receptor complex plays an insignificant role.
GP Ibbeta/GP IX (zeige GP9 ELISA Kits) mediates the disulfide-linked GP Ibalpha localization to the GEMs, which is critical for vWf (zeige VWF ELISA Kits) interaction at high shear
Data show that the surface-bound VWF (zeige VWF ELISA Kits) appears as a large, linear structure on the surface of 50% of the PT-VWD (zeige VWF ELISA Kits) platelets.
Role of GPIbbeta in modulating vWF (zeige VWF ELISA Kits) mediated platelet adhesion.
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.
glycoprotein Ib (platelet), beta polypeptide
, GP-Ib beta
, antigen CD42b-beta
, nuclear localization signal deleted in velocardiofacial syndrome
, platelet glycoprotein Ib beta chain
, glycoprotein Ib, beta polypeptide