4.5 Review

The human complement factor H:: functional roles, genetic variations and disease associations

Journal

MOLECULAR IMMUNOLOGY
Volume 41, Issue 4, Pages 355-367

Publisher

PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.molimm.2004.02.005

Keywords

factor H; complement; RCA; hemolytic-uremic-syndrome; HUS

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Factor H is an essential regulatory protein that plays a critical role in the homeostasis of the complement system in plasma and in the protection of bystander host cells and tissues from damage by complement activation. Genetic and structural data generated during recent years have been instrumental to delineate the functional domains responsible for these regulatory activities in factor H, which is helping to understand the molecular basis underlying the different pathologies associated to factor H. This review summarises our current knowledge of the role of factor H in health and disease. (C) 2004 Published by Elsevier Ltd.

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