Article
Hemophilia A mutations within the factor VIII A2-A3 subunit interface destabilize factor VIIIa and cause one-stage/two-stage activity discrepancy.
Thrombosis and haemostasis - 1 Nov 2002
Hakeos William H, Miao Hongzhi, Sirachainan Nongnuch, Kemball-Cook Geoffrey, Saenko Evgueni L, Kaufman Randal J, Pipe Steven W
Abstract excerpt
Thrombin-activated factor VIII (FVIIIa) is a heterotrimer with the A2 subunit in a weak ionic interaction with the A1 and A3-C1-C2 subunits. Dissociation of the A2 subunit correlates with inactivation of FVIIIa. A homology model (Blood 89:2413, 1997) of the triplicated A domains of factor VIII (FVIII) predicts a pseudo-threefold axis at the tightly packed hydrophobic core with several interdomain interactions....
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