Control of von Willebrand factor multimer size and implications for disease

Plasma von Willebrand factor (vWF) is a multimeric protein that mediates adhesion of platelets to sites of vascular injury, however only the very large vWF multimers are haemostatically competent. Plasma vWF derives predominantly from the vascular endothelium and is of a smaller average multimer siz...

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Bibliographic Details
Published in:Blood reviews 2002-09, Vol.16 (3), p.185-192
Main Authors: Pimanda, John, Hogg, Philip
Format: Article
Language:English
Subjects:
ADAM Proteins
ADAMTS13 Protein
Biological and medical sciences
Blood coagulation. Blood cells
Coagulation factors
Dimerization
Fundamental and applied biological sciences. Psychology
haemolytic uraemic syndrome
Humans
Metalloendopeptidases - metabolism
Metalloendopeptidases - physiology
Molecular and cellular biology
Thrombosis - etiology
Thrombosis - prevention & control
thrombospondin
Thrombospondin 1 - metabolism
Thrombospondin 1 - physiology
thrombotic thrombocytopaenic purpura
von Willebrand factor
von Willebrand Factor - metabolism
von Willebrand Factor - physiology
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Summary:Plasma von Willebrand factor (vWF) is a multimeric protein that mediates adhesion of platelets to sites of vascular injury, however only the very large vWF multimers are haemostatically competent. Plasma vWF derives predominantly from the vascular endothelium and is of a smaller average multimer size to that found in the sub-endothelial matrix. The existence of plasma factors that control the size of vWF multimers and account for this difference has long been suspected. vWF cleaving protease (vWFCP), a metalloproteinase that regulates vWF multimer size by proteolytic cleavage, and thrombospondin-1 (TSP-1), a trimeric glycoprotein that uncouples multimers by reducing the disulfide-bonds which link individual subunits, are two such factors that have recently been identified. The large and ultra large vWF multimers play a central role in arterial thrombosis and agents that regulate their size hold promise as novel anti-thrombotic drugs.
ISSN:0268-960X
1532-1681
DOI:10.1016/S0268-960X(02)00017-6