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首页>>免疫学>>一抗>>凝血因子8/第八凝血因子/第八因子相关抗原抗体
凝血因子8/第八凝血因子/第八因子相关抗原抗体
  • 产品货号：
    BN40250R
  • 中文名称：
    凝血因子8/第八凝血因子/第八因子相关抗原抗体
  • 英文名称：
    Rabbit anti-Factor VIII B chain Polyclonal antibody
  • 品牌：
    Biorigin

  • 货号

    产品规格

    售价

    备注

  • BN40250R-100ul

    100ul

    ¥2360.00

    交叉反应：Human 推荐应用：WB,IHC-P,IHC-F,IF,ELISA

  • BN40250R-200ul

    200ul

    ¥3490.00

    交叉反应：Human 推荐应用：WB,IHC-P,IHC-F,IF,ELISA

产品描述

英文名称Factor VIII B chain
中文名称凝血因子8/第八凝血因子/第八因子相关抗原抗体
别    名coagulation factor VIII; Ahf; Antihemophilic factor; Coagulation factor VIII; Coagulation factor VIII associated protein b; Coagulation factor VIII isoform b; Coagulation factor VIII procoagulent component; Coagulation factor VIIIc; Dna segment on chromosome x unique 1253 expressed sequence; Dxs1253e; F8; F8 protein; F8b; F8c; Factor VIII F8b; FactorVIII; FVIII; Hema coagulation factor VIIIc procoagulent component; Hemophilia a; Hemophilia classic; OTTHUMP00000061446; Procoagulant component; FⅧ-Ag; Factor VIIIa heavy chain, 200 kDa isoform; Factor VIIIa heavy chain, 92 kDa isoform; Factor VIII B chain; AHF; DXS1253E; F8B; F8C; FVIII; HEMA; FA8_HUMAN.  
研究领域肿瘤  心血管  免疫学  神经生物学  
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, 
产品应用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=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138kDa
细胞定位细胞外基质 分泌型蛋白 
性    状Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human Factor VIII B chain:1101-1200/2351 
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Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
产品介绍This gene encodes coagulation factor VIII, which participates in the intrinsic pathway of blood coagulation; factor VIII is a cofactor for factor IXa which, in the presence of Ca+2 and phospholipids, converts factor X to the activated form Xa. This gene produces two alternatively spliced transcripts. Transcript variant 1 encodes a large glycoprotein, isoform a, which circulates in plasma and associates with von Willebrand factor in a noncovalent complex. This protein undergoes multiple cleavage events. Transcript variant 2 encodes a putative small protein, isoform b, which consists primarily of the phospholipid binding domain of factor VIIIc. This binding domain is essential for coagulant activity. Defects in this gene results in hemophilia A, a common recessive X-linked coagulation disorder. [provided by RefSeq, Jul 2008].

Function:
Factor VIII, along with calcium and phospholipid, acts as a cofactor for factor IXa when it converts factor X to the activated form, factor Xa.

Subunit:
Interacts with vWF. vWF binding is essential for the stabilization of F8 in circulation.

Subcellular Location:
Secreted, extracellular space.

Post-translational modifications:
Sulfation on Tyr-1699 is essential for binding vWF.

DISEASE:
Hemophilia A (HEMA) [MIM:306700]: A disorder of blood coagulation characterized by a permanent tendency to hemorrhage. About 50% of patients have severe hemophilia resulting in frequent spontaneous bleeding into joints, muscles and internal organs. Less severe forms are characterized by bleeding after trauma or surgery. Note=The disease is caused by mutations affecting the gene represented in this entry. Of particular interest for the understanding of the function of F8 is the category of CRM (cross-reacting material) positive patients (approximately 5%) that have considerable amount of F8 in their plasma (at least 30% of normal), but the protein is non-functional; i.e. the F8 activity is much less than the plasma protein level. CRM-reduced is another category of patients in which the F8C antigen and activity are reduced to approximately the same level. Most mutations are CRM negative, and probably affect the folding and stability of the protein.

Similarity:
Belongs to the multicopper oxidase family.
Contains 3 F5/8 type A domains.
Contains 2 F5/8 type C domains.
Contains 6 plastocyanin-like domains.

SWISS:
P00451

Gene ID:
2157

Database links:

Entrez Gene: 403875 Dog

Entrez Gene: 2157 Human

Entrez Gene: 14069 Mouse

Entrez Gene: 397339 Pig

Omim: 300841 Human

SwissProt: O18806 Dog

SwissProt: P00451 Human

SwissProt: Q06194 Mouse

SwissProt: P12263 Pig

Unigene: 654450 Human

Unigene: 1805 Mouse



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