A mutant complement factor H (W1183R) enhances proteolytic cleavage of von Willebrand factor by ADAMTS-13 under shear

These results demonstrate for the first time that W1183R-CFH but not WT-CFH protein enhances the proteolytic cleavage of VWF by ADAMTS-13 under shear. This may be achieved by mechanic-induced conformational changes of the central A2 domain, leading to an enhanced cleavage of Tyr<sup>1605</sup>-Met<sup>1606</sup> bond by ADAMTS-13 under pathophysiological conditions.

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Publication Details

Journal
Journal of Thrombosis and Haemostasis
Published
2025-01-09
DOI
https://doi.org/10.1016/j.jtha.2024.11.031
Citations
2
Primary Topic
Complement system in diseases
Type
article
Field-Weighted Citation Impact
1.59

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article

A mutant complement factor H (W1183R) enhances proteolytic cleavage of von Willebrand factor by ADAMTS-13 under shear

X. Long Zheng, Yi Liu, Xiao Hui Zhang, Wenjing Cao
2 citations
Journal of Thrombosis and Haemostasis
Complement system in diseases
1.59
article

A mutant complement factor H (W1183R) enhances proteolytic cleavage of von Willebrand factor by ADAMTS-13 under shear

X. Long Zheng, Yi Liu, Xiao Hui Zhang, Wenjing Cao
article en
2 citations

Abstract

These results demonstrate for the first time that W1183R-CFH but not WT-CFH protein enhances the proteolytic cleavage of VWF by ADAMTS-13 under shear. This may be achieved by mechanic-induced conformational changes of the central A2 domain, leading to an enhanced cleavage of Tyr<sup>1605</sup>-Met<sup>1606</sup> bond by ADAMTS-13 under pathophysiological conditions.

Journal of Thrombosis and HaemostasisVol. 23(4)
University of Massachusetts Amherst (US), University of Kansas Medical Center (US)
National Heart, Lung, and Blood Institute
Zero hunger
Openalex Percentile: Top 20%
Complement system in diseases
1.59
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