Homology models of FV A2 and A3 domains showing the position of the 2 identified missense mutations. Ribbon diagrams of human FV A2 (left panel) and A3 (right panel) domain homology models were produced using SwissPDB 3.7 software and the coordinates under Protein Data Bank entry 1FV4.⁹  In each panel, the N- and C-terminal β-barrels and the amino(N) and carboxy-termini (C) of the domain are indicated. Each A domain is oriented to best display the sites of mutated residues (Cys472 and Val1813). The position of Cys498, involved in a disulfide bridge with Cys472 in the wild-type protein, is also indicated.