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Factor H-Related Proteins

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The Complement System

Part of the book series: Methods in Molecular Biology ((MIMB,volume 1100))

Abstract

Factor H-related proteins (CFHRs) are plasma glycoproteins related in structure and antigenicity to each other and to the complement inhibitory protein factor H. Such proteins are found in most mammals but their number and domain composition vary. This chapter summarizes our current knowledge on the human factor H-related proteins. In contrast to factor H, they have no strong complement inhibitory activity, although for some of them regulatory or complement modulatory activity has been reported. A common feature of CFHRs is that they bind to the C3b component of complement. Novel links between CFHRs and various diseases (C3 glomerulopathies, atypical hemolytic uremic syndrome and age-related macular degeneration) have been revealed in recent years, but we are still far from understanding their biological function.

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Acknowledgements

M.J. is supported by the “Lendület” Program of the Hungarian Academy of Sciences (grant LP2012-43/2012). S.M. is supported by the Sigrid Jusélius Foundation, Helsinki University Central Hospital Funds and the Immunobiology Research Program, University of Helsinki.

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Authors and Affiliations

  1. Junior Research Group Cellular Immunobiology, Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany

    Mihály Józsi

  2. MTA-ELTE “Lendületc” Complement Research Group, Department of Immunology, Eötvös Loránd University, Budapest, Hungary

    Mihály Józsi

  3. Department of Bacteriology and Immunology, Haartman Institute, University of Helsinki, Helsinki, Finland

    Seppo Meri

Authors
  1. Mihály Józsi
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  2. Seppo Meri
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Editors and Affiliations

  1. Division of Infectious Diseases, Brigham and Women’s Hospital, Harvard University Harvard Medical School, Boston, Massachusetts, USA

    Mihaela Gadjeva

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Józsi, M., Meri, S. (2014). Factor H-Related Proteins. In: Gadjeva, M. (eds) The Complement System. Methods in Molecular Biology, vol 1100. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-724-2_18

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  • DOI: https://doi.org/10.1007/978-1-62703-724-2_18

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  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-62703-723-5

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