The role of heparan sulfate as determining pathogenic factor in complement factor H-associated diseases
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Publication year
2015Source
Molecular Immunology, 63, 2, (2015), pp. 203-8ISSN
Publication type
Article / Letter to editor
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Organization
Nephrology
Journal title
Molecular Immunology
Volume
vol. 63
Issue
iss. 2
Page start
p. 203
Page end
p. 8
Subject
Radboudumc 11: Renal disorders RIMLS: Radboud Institute for Molecular Life SciencesAbstract
Complement factor H (FH) systemically inhibits excessive complement activation in the microenvironment of host cells, but for instance not on microbes. This self-recognition is mediated by two binding sites that recognize distinctly sulfated heparan sulfate (HS) domains. The interaction with HS not only concentrates FH on host cells, but directly affects its activity, evoking novel models of conformational activation. Genetic aberrations in the HS-binding domains systemically disturb the protective function of FH, yet the resulting loss of complement control affects mainly ocular and renal tissues. Recent results suggest that the specific expression of HS domains in these tissues restricts the interaction of HS to a single binding site within FH. This lack of redundancy could predispose eyes and kidneys to complement-mediated damage, making HS a central determinant for FH-associated diseases.
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- Faculty of Medical Sciences [90373]
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