The Lancet 1976-Apr
Molecular defects in haemophilia A and von Willebrand's disease.
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Mots clés
Abstrait
A model is proposed for the synthesis of the factor VII/von Willebrand factor protein. The defect(s) in von Willebrand's disease are related to abnormalities of the protein subunit and/or its carbohydrate content, while in haemophilia the abnormality is related to a deficiency or abnormality of the X-dependent enzyme or protein which is involved in the generation of procoagulant activity of the factor VII/von Willebrand factor multimer.