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GP1BA Recombinant Rabbit mAb (bsm-60018R)  
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產品編號 bsm-60018R
英文名稱 GP1BA Recombinant Rabbit mAb
中文名稱 血小板糖蛋白GPIb(CD42b)重組兔單抗
別    名 Antigen CD42b alpha; BSS; CD 42b; CD42b alpha; CD42b antigen; GLYCOCALICIN; Glycoprotein Ib(platelet) alpha polypeptide; Glycoprotein Ibalpha; GP Ib alpha; GP1B; CD42b; GPIb alpha; MGC34595; Platelet glycoprotein Ib alpha chain; Platelet glycoprotein Ib a  
研究領域 心血管  細胞生物  免疫學  細胞粘附分子  細胞表面分子  
抗體來源 Rabbit
克隆類型 Recombinant
交叉反應 Human
產品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 67 kDa
檢測分子量
細胞定位 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GP1BA/CD42b 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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. Several polymorphisms and mutations have been described in this gene, some of which are the cause of Bernard-Soulier syndromes and platelet-type von Willebrand disease. [provided by RefSeq, Mar 2010].

Function:
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.

Subunit:
Heterodimer composed of GP-Ib alpha and beta; disulfide linked. GP-IX is complexed with the GP-Ib heterodimer via a non covalent linkage. Interacts with FLNB.

Subcellular Location:
Membrane; Single-pass type I membrane protein.

Post-translational modifications:
lycocalicin, which is approximately coextensive with the extracellular part of the molecule, is cleaved off by calpain during platelet lysis.

DISEASE:
Genetic variations in GP1BA may be a cause of susceptibility to non-arteritic anterior ischemic optic neuropathy (NAION) [MIM:258660]. 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) [MIM:231200]; 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) [MIM:153670]; 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 von Willebrand disease platelet-type (PVWD) [MIM:177820]; also known as pseudo-von Willebrand disease (pseudo-vWD). This autosomal dominant 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.

Similarity:
Contains 7 LRR (leucine-rich) repeats.
Contains 1 LRRCT domain.
Contains 1 LRRNT domain.

SWISS:
P07359

Gene ID:
2811

Database links:

Entrez Gene: 2811 Human

Omim: 606672 Human

SwissProt: P07359 Human

Unigene: 1472 Human



產品圖片
Paraformaldehyde-fixed, paraffin embedded Human Spleen; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with GP1BA Monoclonal Antibody, Unconjugated(bsm-60018R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
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