| 產(chǎn)品編號 | bs-0332R-BF594 |
| 英文名稱 | Rabbit Anti-factor VIII/BF594 Conjugated antibody |
| 中文名稱 | BF594標(biāo)記的凝血因子8/第八凝血因子/第八因子相關(guān)抗原抗體 |
| 別 名 | FVIII; 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; Hema coagulation factor VIIIc procoagulent component; Hemophilia a; Hemophilia classic; OTTHUMP00000061446; Procoagulant component; AHF; DXS1253E; F8B; F8C; FVIII; HEMA; FA8_HUMAN. |
| 規(guī)格價格 | 100ul/2980元 購買 大包裝/詢價 |
| 說 明 書 | 100ul |
| 研究領(lǐng)域 | 腫瘤 心血管 免疫學(xué) 神經(jīng)生物學(xué) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human, |
| 產(chǎn)品應(yīng)用 | IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 230kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human factor VIII |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | 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. |
| 產(chǎn)品介紹 |
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: 2157 Human Omim: 300841 Human SwissProt: P00451 Human Unigene: 654450 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. Factor VIII-related Antigen （又稱：凝結(jié)因子VIII,抗血友病因子）第VIII因子抗體用于血管源性良性和惡性腫瘤的診斷，也用于遺傳性血友病的研究。在正常動脈、靜脈、毛細(xì)血管及心臟內(nèi)細(xì)胞的血管內(nèi)皮細(xì)胞上陽性表達。在巨核細(xì)胞及血小板上也有表達。 第八因子相關(guān)抗原-Ⅷ因子抗體:是一種糖蛋白，廣泛存在于血管上皮、肝臟、脾竇上皮、及淋巴內(nèi)皮細(xì)胞，是血管內(nèi)皮細(xì)胞及其內(nèi)源性良惡性腫瘤的特異性標(biāo)記。主要用于血管原性良惡性腫瘤和血管肉瘤的診斷。少數(shù)副睪、子宮和輸卵管的腺癌樣瘤也有表達. |
