| 產(chǎn)品編號(hào) | bs-0434R |
| 英文名稱 | factor VIII Rabbit pAb |
| 中文名稱 | 凝血因子8/第八凝血因子/第八因子相關(guān)抗原抗體 |
| 別 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. |
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Specific References (11) | bs-0434R has been referenced in 11 publications.
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| 研究領(lǐng)域 | 腫瘤 免疫學(xué) 生長(zhǎng)因子和激素 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human,Mouse,Rat (predicted: Rabbit,Dog) |
| 產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 267 kDa |
| 檢測(cè)分子量 | |
| 細(xì)胞定位 | 細(xì)胞外基質(zhì) 分泌型蛋白 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from rat factor VIII: 401-500/2258 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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 Entrez Gene: 14069 Mouse Omim: 300841 Human SwissProt: P00451 Human SwissProt: Q06194 Mouse Unigene: 654450 Human Unigene: 1805 Mouse
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(dòng)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(dá)。第Ⅷ因子相關(guān)抗原抗體在巨核細(xì)胞及血小板上也有表達(dá)。 第Ⅷ因子相關(guān)抗原: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(dá). |
| 產(chǎn)品圖片 |
Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-Factor VIII Polyclonal Antibody, Unconjugated(bs-0434R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Paraformaldehyde-fixed, paraffin embedded (rat brain tissue); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (factor VIII) Polyclonal Antibody, Unconjugated (bs-0434R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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