Factor VIII B chain Rabbit pAb (一抗) | Bioss
货号：bs-10397R
产品详情
相关标记
相关产品
相关文献
常见问题
概述
产品编号
bs-10397R
英文名称
Factor VIII B chain Rabbit pAb
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
英文别名
AHF; DXS1253E; F8B; F8C; FVIII; HEMA; THPH13; Cf-8; Cf8; FA8_CANLF; F8; Procoagulant component; FA8_HUMAN; Antihemophilic factor (AHF); FA8_MOUSE; FA8_PIG; coagulation factor VIII; coagulation factor VIII, procoagulant component; Factor VIIIF8B; hemophilia A
抗体来源
Rabbit
免疫原
KLH conjugated synthetic peptide derived from human Factor VIII B chain: 1101-1200/2351
亚型
IgG
性状
Liquid
纯化方法
affinity purified by Protein A
克隆类型
Polyclonal
理论分子量
138 kDa
检测分子量
110 kDa
浓度
1mg/ml
储存液
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
研究领域
SWISS
Gene ID
保存条件
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
数据库链接
产品介绍
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、毛细血管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、及淋巴内皮细胞，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、子宫和输卵管的腺癌样瘤也有表达.
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、及淋巴内皮细胞，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、子宫和输卵管的腺癌样瘤也有表达.
背景资料
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].
产品应用
| 应用 | 已检合格种属 | 预测种属 | 推荐稀释比例 |
|---|---|---|---|
| WB | Human | 1:500-2000 | |
| IHC-P | Human | 1:100-500 | |
| IHC-F | Human | 1:100-500 | |
| IF | Human | 1:100-500 |
交叉反应
交叉反应: Human
相关产品
暂无相关产品
靶标
基因名
F8
蛋白名
Coagulation factor VIII
亚基
Interacts with vWF. vWF binding is essential for the stabilization of F8 in circulation.
亚细胞定位
Secreted, extracellular space.
翻译后修饰
Sulfation on Tyr-1699 is essential for binding vWF.
疾病
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.
相似性
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.
Contains 3 F5/8 type A domains.
Contains 2 F5/8 type C domains.
Contains 6 plastocyanin-like domains.
功能
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.
同靶标产品
相关文献
提示: 发表研究结果有使用 bs-10397R 时请让我们知道，以便我们可以引用参考文章。作为回馈，资料提供者将获得我们送上的小礼品。