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Cysteamine enhances the procoagulant activity of Factor VIII-East Hartford, a dysfunctional protein due to a light chain thrombin cleavage site mutation (arginine-1689 to cysteine).

We have recently identified the molecular defect responsible for cross-reacting material-positive hemophilia A in two unrelated patients in which the substitution of cysteine for arginine-1689 (Factor VIII-East Hartford[FVIII-EH]) abolishes a critical Factor VIII light chain thrombin cleavage site....

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Hlavní autoři: Aly, A M, Arai, M, Hoyer, L W
Médium: Artigo
Jazyk:Inglês
Vydáno: 1992
Témata:
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC443005/
https://ncbi.nlm.nih.gov/pubmed/1569180
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