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Molecular defects in haemophilia A and von Willebrand's disease.

作者信息

Gralnick H R, Coller B S

出版信息

Lancet. 1976 Apr 17;1(7964):837-8. doi: 10.1016/s0140-6736(76)90485-2.

Abstract

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.

摘要

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