Factor H-related proteins determine complement-activating surfaces. [revisión]
Por: Goicoechea de Jorge, Elena [Instituto de Investigación imas12]
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Colaborador(es): Instituto de Investigación imas12.
Tipo de material: 
ArtículoEditor: Trends in immunology, 2015Descripción: 36(6):374-84.Recursos en línea: Solicitar documento Resumen: 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.
| Tipo de ítem | Ubicación actual | Signatura | Estado | Fecha de vencimiento |
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Revisión
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PC17007 (Navegar estantería) | Disponible |
Formato Vancouver:
Józsi M, Tortajada A, Uzonyi B, Goicoechea de Jorge E, Rodríguez de Córdoba S. Factor H-related proteins determine complement-activating surfaces. Trends Immunol. 2015 Jun;36(6):374-84.
PMID: 25979655
Contiene 91 referencias
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.
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