Complement Factor H Related Protein 1 (CFHR1)

CFHL; CFHL1; CFHL1P; CFHR1P; FHR1; H36-1; H36-2; HFL1; HFL2; H factor-like protein 1

SPN	N061
HGNC	4888
MGI	2138169
PubMed	23728178
UniProt	Q03591
Wiki	CFHR1

encodes a secreted protein belonging to the complement factor H protein family. It binds to Pseudomonas aeruginosa elongation factor Tuf together with plasminogen, which is proteolytically activated. It is proposed that Tuf acts as a virulence factor by acquiring host proteins to the pathogen surface, controlling complement, and facilitating tissue invasion. Mutations in this gene are associated with an increased risk of atypical hemolytic-uremic syndrome.

Factor H, via SCR domains 6 to 7 and 19 to 20, and FHR1, via SCR domains 3 to 5, bound to surface-expressed Pseudomonas aeruginosa elongation factor Tuf and also to recombinant Tuf. Factor H and plasminogen (PLG) bound simultaneously to Tuf, and PLG was proteolytically activated.

Reference

"Cloning of the 1.4-kb mRNA species of human complement factor H reveals a novel member of the short consensus repeat family related to the carboxy terminal of the classical 150-kDa molecule."
J. Immunol. 146:3190-3196(1991) [PubMed] [Europe PMC] [Abstract]

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Mol. Immunol. 37:41-52(2000) [PubMed] [Europe PMC] [Abstract]

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Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]

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"Two major serum components antigenically related to complement factor H are different glycosylation forms of a single protein with no factor H-like complement regulatory functions."
J. Immunol. 146:1265-1270(1991) [PubMed] [Europe PMC] [Abstract]

"Complement factor H and related proteins: an expanding family of complement-regulatory proteins?"
Immunol. Today 15:121-126(1994) [PubMed] [Europe PMC] [Abstract]

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"Dimerization of complement factor H-related proteins modulates complement activation in vivo."
Proc. Natl. Acad. Sci. U.S.A. 110:4685-4690(2013) [PubMed] [Europe PMC] [Abstract]