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
产品名称： 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
英文名称： Anti-Factor VIII/FITC
产品编号： HZ-2974R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： IF=1:50-200
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Rabbit Anti-Factor VIII/FITC Conjugated antibody
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
| 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 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. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 心血管 细胞生物 信号转导 细胞表面分子 细胞类型标志物 血管内皮细胞 肿瘤细胞生物标志物 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, 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 | 200/259kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from Rabbit 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. |
| 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]: 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. 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 |
该基因编码凝血因子VIII，参与凝血的内在途径；因子VIII是因子IXA的辅因子，其在Ca+ 2和磷脂存在下，将因子X转换为活化形式XA。该基因产生两个交替剪接转录本。转录变体1编码一个大的糖蛋白，异构体A，它在血浆中循环，并与非共价复合物中的von Willebrand因子相关联。这种蛋白质经历多次解理事件。转录物变体2编码一个假定的小蛋白，异构体B，其主要由因子VIIIc的磷脂结合结构域组成。该结合域对于凝血活性至关重要。该基因的缺陷导致血友病A，这是一种常见的隐性X连锁性凝血障碍。[ RefSeq，JUL 2008 ]提供。