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2018 ; 9
(ä): 1607
Nephropedia Template TP
gab.com Text
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English Wikipedia
Self-Damage Caused by Dysregulation of the Complement Alternative Pathway:
Relevance of the Factor H Protein Family
#MMPMID30050540
Sánchez-Corral P
; Pouw RB
; López-Trascasa M
; Józsi M
Front Immunol
2018[]; 9
(ä): 1607
PMID30050540
show ga
The alternative pathway is a continuously active surveillance arm of the
complement system, and it can also enhance complement activation initiated by the
classical and the lectin pathways. Various membrane-bound and plasma regulatory
proteins control the activation of the potentially deleterious complement system.
Among the regulators, the plasma glycoprotein factor H (FH) is the main inhibitor
of the alternative pathway and its powerful amplification loop. FH belongs to a
protein family that also includes FH-like protein 1 and five factor H-related
(FHR-1 to FHR-5) proteins. Genetic variants and abnormal rearrangements involving
the FH protein family have been linked to numerous systemic and organ-specific
diseases, including age-related macular degeneration, and the renal pathologies
atypical hemolytic uremic syndrome, C3 glomerulopathies, and IgA nephropathy.
This review covers the known and recently emerged ligands and interactions of the
human FH family proteins associated with disease and discuss the very recent
experimental data that suggest FH-antagonistic and complement-activating
functions for the FHR proteins.