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Updating the physiology, exploration and disease relevance of complement factor H.

Abstract : The factor H (FH) protein (also known as beta1H globulin) is the main regulator of the complement alternative pathway. It exhibits multivalent binding sites to the complement component C3b, and polyanions and one binding site to sialic acid and cell surfaces. These multiple binding sites confer to FH a decay-accelerating factor activity in the fluid phase as well as at the cell surface. A defect in FH activity or a FH protein deficiency triggers chronic inflammation and tissue injury, leading to various disorders impacting the kidney or the eye. In contrast, some pathogens, as well as cancer cells, develop various strategies to bind FH and thereby subvert a complement attack. We focus on the functions of FH, and review the main pathological conditions in which FH is involved. Since the pathogenesis is elusive, appropriate FH dosage in biological fluids and FH gene analysis may help in improving understanding of such diseases.
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Contributor : Geneviève Michel Connect in order to contact the contributor
Submitted on : Monday, March 23, 2015 - 2:58:51 PM
Last modification on : Wednesday, September 7, 2022 - 11:32:04 AM


  • HAL Id : hal-01134381, version 1
  • PUBMED : 20646335



V Buhé, Séverine Loisel, J O Pers, K Le Ster, C Berthou, et al.. Updating the physiology, exploration and disease relevance of complement factor H.. International Journal of Immunopathology and Pharmacology, 2010, 23 (2), pp.397-404. ⟨hal-01134381⟩



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