ELK Biotechnology FA8A (heavy chain 200k/92k, Cleaved-Ala20) rabbit pAb

제품명

FA8A (heavy chain 200k/92k, Cleaved-Ala20) rabbit pAb

제품 개요

이 항체는 인간 FA8A 단백질의 heavy chain (200k/92k, Cleaved-Ala20) 부위를 인식하도록 합성된 펩타이드로부터 제작된 토끼 폴리클로날 항체입니다. 혈우병 A 관련 연구 및 응고 인자 VIII 분석에 활용됩니다.

제품 정보

Background

Defects in F8 cause 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.

• Severe hemophilia A: F8C activity <1% of normal, frequent spontaneous bleeding
• Moderately severe: 2–5% activity, bleeding after minor trauma
• Mild: 5–30% activity, bleeding after significant trauma or surgery

CRM-positive patients (~5%) have normal F8C levels but non-functional protein, while CRM-reduced and CRM-negative mutations affect folding and stability.

Functional Notes:

• Factor VIII acts as a cofactor with calcium and phospholipid for Factor IXa to convert Factor X to Factor Xa.
• Domain F5/8 type C2 is essential for phospholipid binding and Factor VIII activity.
• Sulfation on Tyr-1699 is crucial for vWF binding.
• Interacts with vWF for stabilization in circulation.

Similarity:

• Belongs to the multicopper oxidase family
• Contains 1 F5/8 type C domain, 2 F5/8 type C domains, 3 F5/8 type A domains, and 6 plastocyanin-like domains

Pharmaceutical Use:
Available under the names Kogenate (Bayer) and Recombinate (Baxter, American Home Products) for the treatment of hemophilia A.