Anti-GP1BB Antibody (CAB10113)
- SKU:
- CAB10113
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cardiovascular
Frequently bought together:
Description
| Antibody Name: | Anti-GP1BB Antibody |
| Antibody SKU: | CAB10113 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 27-147 of human GP1BB (NP_000398.1). |
| Application: | WB |
| Recommended Dilution: | WB 1:200 - 1:1000 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | LO2, THP-1, Mouse heart, Mouse brain, Rat brain |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 27-147 of human GP1BB (NP_000398.1). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | PAPC SCAG TLVD CGRR GLTW ASLP TAFP VDTT ELVL TGNN LTAL PPGL LDAL PALR TAHL GANP WRCD CRLV PLRA WLAG RPER APYR DLRC VAPP ALRG RLLP YLAE DELR AACA PGPL C |
| Gene ID: | 2812 |
| Uniprot: | P13224 |
| Cellular Location: | Membrane, Single-pass type I membrane protein |
| Calculated MW: | 21kDa/43kDa |
| Observed MW: | 30kDa |
| Synonyms: | GP1BB, BDPLT1, BS, CD42C, GPIBB, GPIbbeta |
| 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. |
| UniProt Protein Function: | GPIbB: 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. Defects in GP1BB 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. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Membrane protein, integral; Cell surface Chromosomal Location of Human Ortholog: 22q11.21 Cellular Component: integral to plasma membrane; plasma membrane Molecular Function:identical protein binding; protein binding; transmembrane receptor activity Biological Process: blood coagulation; blood coagulation, intrinsic pathway; cell adhesion; cell surface receptor linked signal transduction; platelet activation Disease: Bernard-soulier Syndrome |
| NCBI Summary: | 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. [provided by RefSeq, Dec 2010] |
| UniProt Code: | P13224 |
| NCBI GenInfo Identifier: | 4504073 |
| NCBI Gene ID: | 2812 |
| NCBI Accession: | NP_000398.1 |
| UniProt Secondary Accession: | P13224,Q14422, Q8NG40, |
| UniProt Related Accession: | P13224 |
| Molecular Weight: | |
| NCBI Full Name: | platelet glycoprotein Ib beta chain |
| NCBI Synonym Full Names: | glycoprotein Ib platelet beta subunit |
| NCBI Official Symbol: | GP1BB |
| NCBI Official Synonym Symbols: | BS; CD42C; GPIBB; BDPLT1; GPIbbeta |
| NCBI Protein Information: | platelet glycoprotein Ib beta chain |
| UniProt Protein Name: | Platelet glycoprotein Ib beta chain |
| UniProt Synonym Protein Names: | Antigen CD42b-beta; CD_antigen: CD42c |
| Protein Family: | Platelet glycoprotein |
| UniProt Gene Name: | GP1BB |
| UniProt Entry Name: | GP1BB_HUMAN |
 | Western blot analysis of extracts of various cell lines, using GP1BB antibody (CAB10113) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s. |
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