Anti-CD42b抗体Glycoprotein Ib （GP Ib） is a plaet surface membrane glycoprotein composed of a heterodimer, an alpha chain and a beta chain, that is linked by disulfide bonds.

产品编号 byb-2347R
英文名称 Anti-CD42b抗体
中文名称 血小板糖蛋白GPIb抗体
别    名 Antigen CD42b alpha; BSS; CD 42b; CD42b alpha; CD42b antigen; GLYCOCALICIN; Glycoprotein Ib (plaet) alpha polypeptide; Glycoprotein Ibalpha; GP Ib alpha; GP1B; GP1BA; GPIb alpha; MGC34595; Plaet glycoprotein Ib alpha chain; Plaet glycoprotein Ib alpha polypeptide; Plaet membrane glycoprotein 1b alpha subunit.
Anti-CD42b抗体   
说 明 书 0.1ml  0.2ml  
研究领域 心血管  细胞生物  免疫学  细胞粘附分子  细胞表面分子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 67kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human GP1B/CD42b
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed PubMed
产品介绍 background:
Glycoprotein Ib (GP Ib) is a plaet 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 plaet glycoprotein IX and plaet glycoprotein V. The binding of the GP Ib-IX-V complex to VWF facilitates initial plaet adhesion to vascular subendothelium after vascular injury, and also initiates signaling events within the plaet that lead to enhanced plaet 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 plaet-type von Willebrand disease. [provided by RefSeq, Mar 2010].

Function:
GP-Ib, a surface membrane protein of plaets, participates in the formation of plaet 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:
Glycocalicin, which is approximay coextensive with the extracellular part of the molecule, is cleaved off by calpain during plaet 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 plaet disease (GPD). BSS patients have unusually large plaets 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 plaet function, and normal megakaryocyte count. 
Defects in GP1BA are the cause of von Willebrand disease plaet-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.


Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.