Insights into von Willebrand factor proteolysis: clinical implications [Review]

Bowen, Derrick John and Collins, Peter William ORCID: https://orcid.org/0000-0002-6410-1324 2006. Insights into von Willebrand factor proteolysis: clinical implications [Review]. British Journal of Haematology 133 (5) , pp. 457-467. 10.1111/j.1365-2141.2006.06096.x

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Abstract

The proteolysis of von Willebrand factor (VWF) by the recently discovered metalloprotease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin repeats), is a normal processing step in VWF biochemistry. Emerging data indicate that this step may be influenced by a variety of factors, some of which favour increased proteolysis and some of which compromise proteolysis. The former may predispose to bleeding, whilst the latter appears to be the underlying mechanism for thrombotic thrombocytopenic purpura (TTP). The new insights support the concept of "risk" in bleeding, particularly in the case of type 1 von Willebrand disease (VWD), in much the same way that risk is considered in venous thrombosis. This review presents relevant current knowledge of VWF proteolysis by ADAMTS13, and a novel model of how this may be implicated in type 1 VWD is proposed, based on events at the vessel wall at a time of haemostatic challenge.

Item Type:	Article
Date Type:	Publication
Status:	Published
Schools:	Medicine Systems Immunity Research Institute (SIURI)
Subjects:	R Medicine > R Medicine (General)
Uncontrolled Keywords:	von Willebrand factor; von Willebrand disease; ADAMTS13; proteolysis; thrombotic thrombocytopenic purpura
Publisher:	Wiley-Blackwell
ISSN:	0007-1048
Last Modified:	04 Jan 2023 02:14
URI:	https://orca.cardiff.ac.uk/id/eprint/57585

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