Synthesis as Peptide Chain
- FVIII is synthesized as a single chain polypeptide of 2351 amino acids
- A 19-amino acid signal peptide is cleaved by a protease shortly after synthesis
Arrangement of single chain of FVIII
- Domain structure of FVIII is as under:
- A1 – a1 – A2 – a2 – B – a3 – A3 – C1 – C2
- FVIII amino acid sequence suggests that the molecule is composed of
- A triplicated homology region
- A1 domain
- A2 domain
- A3 domain
- A duplicated homology region
- C1 domain
- C2 domain
- Heavily glycosylated B domain
- A1 domain
- Cleavage site (Arg336/337 & 562/563) by APC
- FVIII binds with
- Phospholipids via C1 & C2 domains
- VWF via a3 domain
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