ELK Biotechnology FA8A (light chain, Cleaved-Glu1668) rabbit pAb

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FA8A (light chain, Cleaved-Glu1668) rabbit pAb

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Defects in F8 gene 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.
About 50% of patients have severe hemophilia A with F8C activity less than 1% of normal, leading to 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% and bleeding only after significant trauma or surgery.

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

• Domain F5/8 type C2: Responsible for phospholipid binding, essential for factor VIII activity.
• Function: Factor VIII acts as a cofactor for factor IXa in converting factor X to factor Xa.
• Mass spectrometry: Disulfated, monosulfated, nonsulfated, sulfated, trisulfated (PubMed:10368977).
• Pharmaceutical relevance: Available under names Kogenate (Bayer) and Recombinate (Baxter, American Home Products) for hemophilia A treatment.
• PTM: Sulfation on Tyr-1699 is essential for vWF binding.
• Similarity: Belongs to multicopper oxidase family; contains F5/8 type C and A domains, and plastocyanin-like domains.
• Subunit interaction: Interacts with vWF, essential for stabilization of F8 in circulation.