| 產(chǎn)品編號 | bs-10417R |
| 英文名稱 | Factor VIIIa light chain Rabbit pAb |
| 中文名稱 | 凝血因子8/第八凝血因子/第八因子相關(guān)抗原輕鏈抗體 |
| 別 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; FactorVIII; FVIII; Hema; Hema coagulation factor VIIIc procoagulent component; Hemophilia a; Hemophilia classic; OTTHUMP00000061446; Procoagulant component; AHF; DXS1253E; F8B; F8C; FVIII; HEMA; FA8_HUMAN. |
| 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 |
| 克 隆 號 | |
| 交叉反應(yīng) | Human,Mouse |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 75 kDa |
| 檢測分子量 | |
| 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 human Factor VIIIa light chain: 1751-1850/2351 |
| 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 | 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. SWISS: P00451 Gene ID: 2157 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 |
| 產(chǎn)品圖片 |
Sample:
Raji (human)cell Lysate at 30 ug
Primary: Anti- Factor VIIIa light chain (bs-10417R)at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 75kD
Observed band size: 85kD
Sample:
Lymph node (Mouse) Lysate at 40 ug
Testis (Mouse) Lysate at 40 ug
Primary: Anti-Factor VIIIa light chain (bs-10417R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 75 kD
Observed band size: 75 kD
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