凝血因子8/第八凝血因子/第八因子相关抗原轻链抗体
规格：1mg/1ml
英文名： Factor VIIIa light chain
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 comp
分子量： 75kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Factor V
交叉反应：Human,
细胞定位：细胞外基质 分泌型蛋白
凝血因子8/第八凝血因子/第八因子相关抗原轻链抗体产品介绍：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 凝血因子8/第八凝血因子/第八因子相关抗原轻链抗体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%)凝血因子8/第八凝血因子/第八因子相关抗原轻链抗体 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. 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.
凝血因子8/第八凝血因子/第八因子相关抗原轻链抗体产品应用：WB=1:100-500 ELISA=1:500-1000 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.
研究领域：肿瘤 心血管 **学 神经生物学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid