产品名称：Anti-factor VIII/FITC抗体

Anti-factor VIII/FITC抗体

产品编号YB0434R-FITC

英文名称Anti-factor VIII/FITC

中文名称FITC标记的凝血因子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 procoagulent component; Coagulation factor VIIIc; Dna segment on chromosome x unique 1253 expressed sequence; Dxs1253e; F8; F8 protein;F8c; 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; AHF; DXS1253E; F8C; FVIII; HEMA; FA8_HUMAN.  

Anti-factor VIII/FITC抗体

说 明 书100ul  

研究领域肿瘤  **学  生长因子和**  

抗体来源Rabbit

克隆类型Polyclonal

交叉反应 Human, Mouse, Rat, Dog, Rabbit,

产品应用IF=1:50-200  

not yet tested in other applications.

optimal dilutions/concentrations should be determined by the end user.

分 子 量267kDa

Anti-factor VIII/FITC抗体性    状Lyophilized or Liquid

浓    度1mg/ml

免 疫 原KLH conjugated synthetic peptide derived from rat factor VIII

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.

Anti-factor VIII/FITC抗体

产品介绍background:

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]: Anti-factor VIII/FITC抗体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.

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.

Antigen （又称：凝结因子VIII,抗血友病因子）第VIII因子抗体用于血管源性良性和恶性肿瘤的诊断，也用于遗传性血友病的研究。在正常动脉、静脉、****及心脏内细胞的血管内皮细胞上阳性表达。第Ⅷ因子相关抗原抗体在巨核细胞及血小板上也有表达。

第Ⅷ因子相关抗原:是一种糖蛋白，广泛存在于血管上皮、肝脏、脾窦上皮、及**内皮细胞，是血管内皮细胞及其内源性良恶性肿瘤的特异性标记。主要用于血管原性良恶性肿瘤和血管肉瘤的诊断。少数副睾、**和输卵管的腺癌样瘤也有表达.