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Murine systemic thrombophilia and hemolytic uremic syndrome from a factor H point mutation.

Abstract：

:Complement plays a key role in host defense, but its dysregulation can cause autologous tissue injury. Complement activation is normally controlled by regulatory proteins, including factor H (FH) in plasma and membrane cofactor protein (MCP) on the cell surface. Mutations in FH and MCP are linked to atypical hemolytic uremic syndrome, a type of thrombotic microangiopathy (TMA) that causes renal failure. We describe here that disruption of FH function on the cell surface can also lead to disseminated complement-dependent macrovascular thrombosis. By gene targeting, we introduced a point mutation (W1206R) into murine FH that impaired its interaction with host cells but did not affect its plasma complement-regulating activity. Homozygous mutant mice carrying this mutation developed renal TMA as well as systemic thrombophilia involving large blood vessels in multiple organs, including liver, lung, spleen, and kidney. Approximately 30% of mutant mice displayed symptoms of stroke and ischemic retinopathy, and 48% died prematurely. Genetic deficiency of complement C3 and factor D prevented both the systemic thrombophilia and renal TMA phenotypes. These results demonstrate a causal relationship between complement dysregulation and systemic angiopathy and suggest that complement activation may contribute to various human thrombotic disorders involving both the micro- and macrovasculature.

journal_name

Blood

journal_title

Blood

authors

Ueda Y,Mohammed I,Song D,Gullipalli D,Zhou L,Sato S,Wang Y,Gupta S,Cheng Z,Wang H,Bao J,Mao Y,Brass L,Zheng XL,Miwa T,Palmer M,Dunaief J,Song WC

doi

10.1182/blood-2016-07-728253

subject

Has Abstract

pub_date

2017-03-02 00:00:00

pages

1184-1196

issue

9

eissn

0006-4971

issn

1528-0020

pii

blood-2016-07-728253

journal_volume

129

pub_type

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