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| Reactivity: | Human, Mouse, Rat |
| Applications: | WB, ELISA |
| Host Species: | Rabbit |
| Isotype: | IgG |
| Clonality: | Polyclonal antibody |
| Gene Name: | glycoprotein Ib platelet subunit beta |
| Gene Symbol: | GP1BB |
| Synonyms: | BS; CD42C; GPIBB; BDPLT1; GPIbbeta; GP1BB |
| Gene ID: | 2812 |
| UniProt ID: | P13224 |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 27-147 of human GP1BB (NP_000398.1). |
| Dilution: | WB 1:200-1:1000 |
| Purification Method: | Affinity purification |
| Concentration: | 2.52 mg/ml |
| Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Storage: | Store at -20°C. Avoid freeze / thaw cycles. |
| Documents: | Manual-GP1BB antibody |
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.
Images
 | Western blot analysis of various lysates using GP1BB Rabbit pAb (A10113) at 1:1000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s. |
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