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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| Hauptverfasser: | , , , , , , , , , , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
November 18, 2013
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| In: |
PLOS ONE
Year: 2013, Jahrgang: 8, Heft: 11 |
| ISSN: | 1932-6203 |
| DOI: | 10.1371/journal.pone.0078617 |
| Online-Zugang: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1371/journal.pone.0078617 Verlag, lizenzpflichtig, Volltext: https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0078617 
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| Verfasserangaben: | 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 |
MARC
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| 245 | 1 | 0 | |a Human factor H-related protein 2 (CFHR2) regulates complement activation |c 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 |
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| 520 | |a 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. | ||
| 650 | 4 | |a Complement activation | |
| 650 | 4 | |a Complement inhibitors | |
| 650 | 4 | |a Complement system | |
| 650 | 4 | |a Dimers | |
| 650 | 4 | |a Enzyme-linked immunoassays | |
| 650 | 4 | |a Immune serum | |
| 650 | 4 | |a Macular degeneration | |
| 650 | 4 | |a Recombinant protein purification | |
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| 700 | 1 | |a Kemper, Markus J. |e VerfasserIn |4 aut | |
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| 700 | 1 | |a Skerka, Christine |e VerfasserIn |0 (DE-588)1216431868 |0 (DE-627)1727665090 |0 (DE-576)502821655 |4 aut | |
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