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Trends Immunol. 2015 Jun;36(6):374-84. doi: 10.1016/j.it.2015.04.008. Epub 2015 May 13.

Factor H-related proteins determine complement-activating surfaces.

Author information

1
MTA-ELTE 'Lendület' Complement Research Group, Department of Immunology, Eötvös Loránd University, Budapest, Hungary.
2
Centro de Investigaciones Biológicas, Consejo Superior de Investigaciones Científicas, Madrid, Spain; Centro de Investigación Biomédica en Enfermedades Raras, Madrid, Spain.
3
MTA-ELTE Immunology Research Group, Department of Immunology, Eötvös Loránd University, Budapest, Hungary.
4
Centre for Complement and Inflammation Research (CCIR), Department of Medicine, Imperial College, London, UK; Inmunología, Facultad de Medicina, Universidad Complutense, Madrid 28040, Spain; Hospital 12 de Octubre Institute of Health Research, Madrid, Spain.
5
Centro de Investigaciones Biológicas, Consejo Superior de Investigaciones Científicas, Madrid, Spain; Centro de Investigación Biomédica en Enfermedades Raras, Madrid, Spain. Electronic address: srdecordoba@cib.csic.es.

Abstract

Complement factor H-related proteins (FHRs) are strongly associated with different diseases involving complement dysregulation, which suggests a major role for these proteins regulating complement activation. Because FHRs are evolutionarily and structurally related to complement inhibitor factor H (FH), the initial assumption was that the FHRs are also negative complement regulators. Whereas weak complement inhibiting activities were originally reported for these molecules, recent developments indicate that FHRs may enhance complement activation, with important implications for the role of these proteins in health and disease. We review these findings here, and propose that FHRs represent a complex set of surface recognition molecules that, by competing with FH, provide improved discrimination of self and non-self surfaces and play a central role in determining appropriate activation of the complement pathway.

KEYWORDS:

CFHRs; complement; disease susceptibility; factor H; factor H-related proteins

PMID:
25979655
DOI:
10.1016/j.it.2015.04.008
[Indexed for MEDLINE]

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