ELK Biotechnology FA8B (Cleaved-Ser760) rabbit pAb
Cleaved-Ser760 부위를 인식하는 FA8B rabbit polyclonal antibody로, 인간 및 마우스 시료에 반응합니다. WB 및 ELISA에 적합하며, 고순도 IgG 형태로 제공됩니다. 혈우병 A 관련 연구 및 응고 인자 VIII 분석에 활용됩니다.
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제품명
FA8B (Cleaved-Ser760) rabbit pAb
공급업체
ELK Biotechnology
제품 설명
FA8B (Cleaved-Ser760) rabbit polyclonal antibody는 인간 FA8B 단백질에서 Cleaved-Ser760 부위를 인식하도록 제작된 항체입니다. 혈액 응고 인자 VIII(F8) 관련 연구 및 혈우병 A 병태생리 분석에 유용합니다.
제품 스펙
| 항목 | 내용 |
|---|---|
| Alternative Names | Coagulation factor VIII (Antihemophilic factor; AHF; Procoagulant component) [Cleaved into: Factor VIIIa heavy chain, 200 kDa isoform; Factor VIIIa heavy chain, 92 kDa isoform; Factor VIII B chain; Factor VIIIa light chain] |
| Applications | WB; ELISA |
| Recommended Dilutions | WB 1:1000–2000, ELISA 1:5000–20000 |
| Immunogen | Synthesized peptide derived from human FA8B (Cleaved-Ser760) |
| Host | Rabbit |
| Storage | -20°C / 1 year |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1 mg/ml |
| Observed Band | 100–260 kDa |
| Gene ID (Human) | 2157 |
| Human Swiss-Prot No. | P00451 |
| Cellular Localization | Secreted, extracellular space |
| Species Reactivity | Human; Mouse |
Background
Defects in F8 are the cause of hemophilia A (HEMA) [MIM:306700], a common recessive X-linked coagulation disorder. The frequency of hemophilia A is 1–2 in 10,000 male births across all ethnic groups.
About 50% of patients have severe hemophilia A with F8C activity less than 1% of normal, leading to frequent spontaneous bleeding. Moderate and mild forms show reduced F8C activity and bleeding after minor trauma or surgery.
Functional Notes:
- Factor VIII acts as a cofactor for factor IXa in converting factor X to factor Xa.
- Domain F5/8 type C2 is responsible for phospholipid binding and essential for activity.
- Interacts with vWF; this binding stabilizes F8 in circulation.
Additional Information:
- PTM: Sulfation on Tyr-1699 is essential for vWF binding.
- Similarity: Belongs to the multicopper oxidase family; contains multiple F5/8 type domains.
- Pharmaceutical relevance: Available as Kogenate (Bayer) and Recombinate (Baxter, AHP) for hemophilia A treatment.
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