Human factor H-related protein 2 (CFHR2) regulates complement activation.

Mutations and deletions within the human CFHR gene cluster on chromosome 1 are associated with diseases, such as dense deposit disease, CFHR nephropathy or age-related macular degeneration. Resulting mutant CFHR proteins can affect complement regulation. Here we identify human CFHR2 as a novel alter...

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Autores principales:	Hannes U Eberhardt, Denise Buhlmann, Peter Hortschansky, Qian Chen, Sascha Böhm, Markus J Kemper, Reinhard Wallich, Andrea Hartmann, Teresia Hallström, Peter F Zipfel, Christine Skerka
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2013
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/0a3e82dd6e944352ab29effbc719de8a
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spelling	oai:doaj.org-article:0a3e82dd6e944352ab29effbc719de8a2021-11-18T08:45:49ZHuman factor H-related protein 2 (CFHR2) regulates complement activation.1932-620310.1371/journal.pone.0078617https://doaj.org/article/0a3e82dd6e944352ab29effbc719de8a2013-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/24260121/pdf/?tool=EBIhttps://doaj.org/toc/1932-6203Mutations and deletions within the human CFHR gene cluster on chromosome 1 are associated with diseases, such as dense deposit disease, CFHR nephropathy or age-related macular degeneration. Resulting mutant CFHR proteins can affect complement regulation. Here we identify human CFHR2 as a novel alternative pathway complement regulator that inhibits the C3 alternative pathway convertase and terminal pathway assembly. CFHR2 is composed of four short consensus repeat domains (SCRs). Two CFHR2 molecules form a dimer through their N-terminal SCRs, and each of the two C-terminal ends can bind C3b. C3b bound CFHR2 still allows C3 convertase formation but the CFHR2 bound convertases do not cleave the substrate C3. Interestingly CFHR2 hardly competes off factor H from C3b. Thus CFHR2 likely acts in concert with factor H, as CFHR2 inhibits convertases while simultaneously allowing factor H assisted degradation by factor I.Hannes U EberhardtDenise BuhlmannPeter HortschanskyQian ChenSascha BöhmMarkus J KemperReinhard WallichAndrea HartmannTeresia HallströmPeter F ZipfelChristine SkerkaPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 8, Iss 11, p e78617 (2013)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Hannes U Eberhardt Denise Buhlmann Peter Hortschansky Qian Chen Sascha Böhm Markus J Kemper Reinhard Wallich Andrea Hartmann Teresia Hallström Peter F Zipfel Christine Skerka Human factor H-related protein 2 (CFHR2) regulates complement activation.
description	Mutations and deletions within the human CFHR gene cluster on chromosome 1 are associated with diseases, such as dense deposit disease, CFHR nephropathy or age-related macular degeneration. Resulting mutant CFHR proteins can affect complement regulation. Here we identify human CFHR2 as a novel alternative pathway complement regulator that inhibits the C3 alternative pathway convertase and terminal pathway assembly. CFHR2 is composed of four short consensus repeat domains (SCRs). Two CFHR2 molecules form a dimer through their N-terminal SCRs, and each of the two C-terminal ends can bind C3b. C3b bound CFHR2 still allows C3 convertase formation but the CFHR2 bound convertases do not cleave the substrate C3. Interestingly CFHR2 hardly competes off factor H from C3b. Thus CFHR2 likely acts in concert with factor H, as CFHR2 inhibits convertases while simultaneously allowing factor H assisted degradation by factor I.
format	article
author	Hannes U Eberhardt Denise Buhlmann Peter Hortschansky Qian Chen Sascha Böhm Markus J Kemper Reinhard Wallich Andrea Hartmann Teresia Hallström Peter F Zipfel Christine Skerka
author_facet	Hannes U Eberhardt Denise Buhlmann Peter Hortschansky Qian Chen Sascha Böhm Markus J Kemper Reinhard Wallich Andrea Hartmann Teresia Hallström Peter F Zipfel Christine Skerka
author_sort	Hannes U Eberhardt
title	Human factor H-related protein 2 (CFHR2) regulates complement activation.
title_short	Human factor H-related protein 2 (CFHR2) regulates complement activation.
title_full	Human factor H-related protein 2 (CFHR2) regulates complement activation.
title_fullStr	Human factor H-related protein 2 (CFHR2) regulates complement activation.
title_full_unstemmed	Human factor H-related protein 2 (CFHR2) regulates complement activation.
title_sort	human factor h-related protein 2 (cfhr2) regulates complement activation.
publisher	Public Library of Science (PLoS)
publishDate	2013
url	https://doaj.org/article/0a3e82dd6e944352ab29effbc719de8a
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Human factor H-related protein 2 (CFHR2) regulates complement activation.

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