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Article

Disease-linked mutations in factor H reveal pivotal role of cofactor activity in self-surface-selective regulation of complement activation.

The Journal of biological chemistry - 11 Aug 2017

Kerr Heather, Wong Edwin, Makou Elisavet, Yang Yi, Marchbank Kevin, Kavanagh David, Richards Anna, Herbert Andrew P, Barlow Paul N

Abstract excerpt

Spontaneous activation enables the complement system to respond very rapidly to diverse threats. This activation is efficiently suppressed by complement factor H (CFH) on self-surfaces but not on foreign surfaces. The surface selectivity of CFH, a soluble protein containing 20 complement-control protein modules (CCPs 1-20), may be compromised by disease-linked mutations. However, which of the several functions of...

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Identifiers

PMID: 28637873
DOI: 10.1074/jbc.M117.795088
PMCID: PMC5555194

Genes

CFH

Variants

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