Article
Engineered factor IX variants bypass FVIII and correct hemophilia A phenotype in mice.
Blood - 12 Jan 2012
Milanov Peter, Ivanciu Lacramioara, Abriss Daniela, Quade-Lyssy Patricia, Miesbach Wolfgang, Alesci Sonja, Tonn Torsten, Grez Manuel, Seifried Erhard, Schüttrumpf Jörg
Abstract excerpt
The complex of the serine protease factor IX (FIX) and its cofactor, factor VIII (FVIII), is crucial for propagation of the intrinsic coagulation cascade. Absence of either factor leads to hemophilia, a disabling disorder marked by excessive hemorrhage after minor trauma. FVIII is the more commonly affected protein, either by X-chromosomal gene mutations or in autoimmune-mediated acquired hemophilia. Whereas...
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