Van der Waals surface space–filled model of factor VIII C domains.

C1 (upper panels) and C2 (lower panels) are viewed in similar orientations to Figure 3A-B, with the left series being the “front” and the right, the “back” surface and where the “back” surface corresponds to a 180° rotation about the vertical axis. The domains are color-coded according to element type—carbon atoms are light green; oxygen, red; and nitrogen, blue—except that side chains of residues associated with hemophilia A point mutations have been colored yellow and labeled where visible. Residues that are sites of other hemophilic mutations that are either not shown or not labeled exhibit less than 10% relative solvent accessibility and are defined as buried in the hydrophobic core (Table3).