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Article

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

Journal of thrombosis and haemostasis : JTH - 1 Apr 2025

Cao Wenjing, Liu Yi, Zhang X Frank, Zheng X Long

Abstract excerpt

BACKGROUND: A loss-of-functional mutation (W1183R) in human complement factor H (CFH) is associated with complement-associated hemolytic uremic syndrome; mice carrying a similar mutation (W1206R) in CFH also develop thrombotic microangiopathy but its plasma von Willebrand factor (VWF) multimer sizes were dramatically reduced. The mechanism underlying such a dramatic change in plasma VWF multimer distribution in...

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Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 39798927
DOI: 10.1016/j.jtha.2024.11.031
PMCID: PMC13123425

Genes

CFH
VWF

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 8 Sept 2026.

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