ELK Biotechnology FA8A (heavy chain 92k, Cleaved-Arg759) rabbit pAb

제품명

FA8A (heavy chain 92k, Cleaved-Arg759) rabbit pAb

공급업체

ELK Biotechnology

제품 개요

이 항체는 인간 FA8A 단백질(heavy chain 92k, Cleaved-Arg759) 유래 합성 펩타이드를 면역원으로 사용하여 제작된 토끼 폴리클로날 항체입니다. 혈액 응고 인자 VIII 관련 연구에 적합하며, WB 및 ELISA 분석에 사용할 수 있습니다.

스펙 정보

Background

Defects in F8 are the cause of hemophilia A (HEMA) [MIM:306700]. HEMA is a common recessive X-linked coagulation disorder with a frequency of 1–2 in 10,000 male births across all ethnic groups.
About 50% of patients have severe hemophilia A (F8C activity <1% of normal), resulting in frequent spontaneous bleeding into joints, muscles, and internal organs.
Moderately severe hemophilia A occurs in about 10% of patients (F8C activity 2–5%), with bleeding after minor trauma.
Mild hemophilia A (30–40% of patients) shows F8C activity of 5–30%, with bleeding only after significant trauma or surgery.

CRM (cross-reacting material) positive patients (~5%) have substantial amounts of F8C in plasma (≥30% of normal) but non-functional protein.
CRM-reduced patients show reduced antigen and activity at similar levels.
Most mutations are CRM negative and likely affect protein folding and stability.

Functional information:

• 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.
• Sulfation on Tyr-1699 is essential for binding to vWF.
• Interacts with vWF; this interaction stabilizes F8 in circulation.
• 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 relevance:
Available under the names Kogenate (Bayer) and Recombinate (Baxter and American Home Products) for hemophilia A treatment.