| |  Online-Ressource |
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| Verfasst von: | Eberhardt, Hannes Uwe [VerfasserIn]  |
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| | Buhlmann, Denise [VerfasserIn] |
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| | Hortschansky, Peter [VerfasserIn] |
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| | Chen, Qian [VerfasserIn] |
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| | Böhm, Sascha [VerfasserIn] |
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| | Kemper, Markus J. [VerfasserIn] |
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| | Wallich, Reinhard [VerfasserIn] |
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| | Hartmann, Andrea [VerfasserIn] |
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| | Hallström, Teresia [VerfasserIn] |
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| | Zipfel, Peter F. [VerfasserIn] |
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| | Skerka, Christine [VerfasserIn] |
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| Titel: | Human factor H-related protein 2 (CFHR2) regulates complement activation |
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| Verf.angabe: | 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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| E-Jahr: | 2013 |
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| Jahr: | November 18, 2013 |
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| Fussnoten: | Gesehen am 07.01.2021 |
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| Titel Quelle: | Enthalten in: PLOS ONE |
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| Ort Quelle: | San Francisco, California, US : PLOS, 2006 |
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| Jahr Quelle: | 2013 |
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| Band/Heft Quelle: | 8(2013,11) Artikel-Nummer e78617, 11 Seiten |
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| ISSN Quelle: | 1932-6203 |
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| Abstract: | 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. |
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| DOI: | doi:10.1371/journal.pone.0078617 |
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| URL: | Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.
Volltext ; Verlag: https://doi.org/10.1371/journal.pone.0078617 |
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| | Volltext: https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0078617 |
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| | DOI: https://doi.org/10.1371/journal.pone.0078617 |
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| Datenträger: | Online-Ressource |
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| Sprache: | eng |
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| Sach-SW: | Complement activation |
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| | Complement inhibitors |
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| | Complement system |
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| | Dimers |
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| | Enzyme-linked immunoassays |
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| | Immune serum |
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| | Macular degeneration |
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| | Recombinant protein purification |
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| K10plus-PPN: | 1744195501 |
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| Verknüpfungen: | → Zeitschrift |
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Human factor H-related protein 2 (CFHR2) regulates complement activation / Eberhardt, Hannes Uwe [VerfasserIn]; November 18, 2013 (Online-Ressource)
