At present, the human Factor H protein family represents seven multidomain, multifunctional serum proteins. This group includes the complement and immune regulators Factor H, the Factor H-like protein 1 (FHL-1) and five Factor H-related proteins proteins (FHR-1, -2, -3, -4 and -5). Each is exclusively composed of individually folded protein domains, termed short consensus repeats (SCRs) or complement control modules. Structure-function analyses allowed the localization of the complement regulatory domain of Factor H and FHL-1 in the N-terminal region within SCRs 1–4. In addition, multiple binding sites for C3b, heparin and microbial surface proteins were localized in the N-terminus, within the middle region and also in the C-terminus of Factor H and FHL-1. Recent results show a central role for the C-terminus of Factor H, i.e. SCRs 19–20. These particular domains are conserved in all FHRs identified so far, include contact points for C3b, heparin and microbial surface proteins and represent a ‘hot-spot’ for gene mutations in patients that suffer from the Factor H-associated form of haemolytic uraemic syndrome.
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November 2002
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Conference Article|
November 01 2002
Factor H family proteins: on complement, microbes and human diseases
P. F. Zipfel;
P. F. Zipfel
1
*Hans Knoell Institute for Natural Products Research, Beutenbergstrasse I la, D-07745 Jena, Germany
1To whom correspondence should be addressed at Department of Infection Biology, Hans Knoell Institute for Natural Products Research, Beutenbergstrasse 11a, D-07745 Jena, Germany (e-mail zipfel@pmail.hki-jena.de)
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C. Skerka;
C. Skerka
*Hans Knoell Institute for Natural Products Research, Beutenbergstrasse I la, D-07745 Jena, Germany
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J. Hellwage;
J. Hellwage
*Hans Knoell Institute for Natural Products Research, Beutenbergstrasse I la, D-07745 Jena, Germany
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S. T. Jokiranta;
S. T. Jokiranta
†Department of Bacteriology and Immunology, Haartman Institute, Haartmaninkatu 3, University of Helsinki, FIN-00290 Helsinki, Finland
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S. Meri;
S. Meri
†Department of Bacteriology and Immunology, Haartman Institute, Haartmaninkatu 3, University of Helsinki, FIN-00290 Helsinki, Finland
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V. Brade;
V. Brade
‡Institute of Medical Microbiology, Frankfurt University Hospital, Paul-Ehrlich-Str. 40, D-60596 Frankfurt, Germany
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P. Kraiczy;
P. Kraiczy
‡Institute of Medical Microbiology, Frankfurt University Hospital, Paul-Ehrlich-Str. 40, D-60596 Frankfurt, Germany
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M. Noris;
M. Noris
§Mario Negri Institute for Pharmacological Research, Bergamo, Italy
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G. Remuzzi
G. Remuzzi
§Mario Negri Institute for Pharmacological Research, Bergamo, Italy
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Publisher: Portland Press Ltd
Received:
June 19 2002
Online ISSN: 1470-8752
Print ISSN: 0300-5127
© 2002 Biochemical Society
2002
Biochem Soc Trans (2002) 30 (6): 971–978.
Article history
Received:
June 19 2002
Citation
P. F. Zipfel, C. Skerka, J. Hellwage, S. T. Jokiranta, S. Meri, V. Brade, P. Kraiczy, M. Noris, G. Remuzzi; Factor H family proteins: on complement, microbes and human diseases. Biochem Soc Trans 1 November 2002; 30 (6): 971–978. doi: https://doi.org/10.1042/bst0300971
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