Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy

24 p.-3 fig.-3 tab.

Detalhes bibliográficos
Autores: Corvillo, Fernando, Bravo García-Morato, María, Nozal, Pilar, Garrido, Sofía, Tortajada, Agustín, Rodríguez de Córdoba, Santiago, López-Trascasa, Margarita
Formato: artículo
Estado:Versión aceptada para publicación
Fecha de publicación:2016
País:España
Recursos:Consejo Superior de Investigaciones Científicas (CSIC)
Repositorio:DIGITAL.CSIC. Repositorio Institucional del CSIC
OAI Identifier:oai:digital.csic.es:10261/138990
Acesso em linha:http://hdl.handle.net/10261/138990
Access Level:acceso abierto
Palavra-chave:Alternative pathway
C3 nephritic factor
C3 glomerulopathy
Complement
C3 glomerulonephritis
Properdin
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spelling Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathyProperdin in C3 glomerulopathyCorvillo, FernandoBravo García-Morato, MaríaNozal, PilarGarrido, SofíaTortajada, AgustínRodríguez de Córdoba, SantiagoLópez-Trascasa, MargaritaAlternative pathwayC3 nephritic factorC3 glomerulopathyComplementC3 glomerulonephritisProperdin24 p.-3 fig.-3 tab.Properdin (P) stabilizes the alternative pathway (AP) convertases, being the only known positive regulator of complement system. In addition, P is a pattern recognition molecule able to initiate directly the AP on non-self surfaces. Although P deficiencies are long time known to be associated with Neisseria infections and P is often found deposited at sites of AP activation and tissue injury, the potential role of P in the pathogenesis of complement dysregulation associated disorders has not been extensively studied. Serum P levels were measured in 49 patients with histological and clinical evidence of C3 glomerulopathy (C3G). Patients were divided in two groups according to the presence or absence of C3 nephritic factor (C3NeF), an autoantibody that stabilizes the AP C3 convertase. The presence of this autoantibody results in a significant reduction in circulating C3 (p<0.001) and C5 levels (p<0.05), but does not alter factor B, P and sC5b-9 levels. Interestingly, in our cohort, serum P levels were low in 17 of the 32 C3NeF-negative patients. This group exhibited significant reduction of C3 (p<0.001) and C5 (p<0.001), and increase of sC5b-9 (p<0.001) plasma levels, compared to the control group. Besides, P consumption was significantly correlated with C3 (r= 0.798, p 0.0001), C5 (r= 0.806, p<0.0001), sC5b-9 (r = -0.683, p 0.043) and a higher degree of proteinuria (r= -0.862, p 0.013). These results further illustrate the heterogeneity among C3G patients and suggest that P serum levels could be a reliable clinical biomarker to identify patients with underlying surface AP C5 convertase dysregulation.This work was performed with the support of grants from the Spanish Ministerio de Economía y Competitividad (SAF2012-38636), Comunidad de Madrid (S2010/BMD-2316) and CIBERER (ACCI-2014).Peer reviewedJohn Wiley & SonsMinisterio de Economía y Competitividad (España)Comunidad de MadridConsejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]201620162016info:eu-repo/semantics/articlehttp://purl.org/coar/resource_type/c_6501Postprintinfo:eu-repo/semantics/acceptedVersionhttp://hdl.handle.net/10261/138990reponame:DIGITAL.CSIC. Repositorio Institucional del CSICinstname:Consejo Superior de Investigaciones Científicas (CSIC)Ingléshttp://dx.doi.org/ 10.1111/cei.12754Síinfo:eu-repo/semantics/openAccessoai:digital.csic.es:10261/1389902026-05-22T06:33:51Z
dc.title.none.fl_str_mv Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy
Properdin in C3 glomerulopathy
title Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy
spellingShingle Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy
Corvillo, Fernando
Alternative pathway
C3 nephritic factor
C3 glomerulopathy
Complement
C3 glomerulonephritis
Properdin
title_short Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy
title_full Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy
title_fullStr Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy
title_full_unstemmed Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy
title_sort Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy
dc.creator.none.fl_str_mv Corvillo, Fernando
Bravo García-Morato, María
Nozal, Pilar
Garrido, Sofía
Tortajada, Agustín
Rodríguez de Córdoba, Santiago
López-Trascasa, Margarita
author Corvillo, Fernando
author_facet Corvillo, Fernando
Bravo García-Morato, María
Nozal, Pilar
Garrido, Sofía
Tortajada, Agustín
Rodríguez de Córdoba, Santiago
López-Trascasa, Margarita
author_role author
author2 Bravo García-Morato, María
Nozal, Pilar
Garrido, Sofía
Tortajada, Agustín
Rodríguez de Córdoba, Santiago
López-Trascasa, Margarita
author2_role author
author
author
author
author
author
dc.contributor.none.fl_str_mv Ministerio de Economía y Competitividad (España)
Comunidad de Madrid
Consejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]
dc.subject.none.fl_str_mv Alternative pathway
C3 nephritic factor
C3 glomerulopathy
Complement
C3 glomerulonephritis
Properdin
topic Alternative pathway
C3 nephritic factor
C3 glomerulopathy
Complement
C3 glomerulonephritis
Properdin
description 24 p.-3 fig.-3 tab.
publishDate 2016
dc.date.none.fl_str_mv 2016
2016
2016
dc.type.none.fl_str_mv info:eu-repo/semantics/article
http://purl.org/coar/resource_type/c_6501
Postprint
info:eu-repo/semantics/acceptedVersion
format article
status_str acceptedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/10261/138990
url http://hdl.handle.net/10261/138990
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv http://dx.doi.org/ 10.1111/cei.12754
Sí
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv John Wiley & Sons
publisher.none.fl_str_mv John Wiley & Sons
dc.source.none.fl_str_mv reponame:DIGITAL.CSIC. Repositorio Institucional del CSIC
instname:Consejo Superior de Investigaciones Científicas (CSIC)
instname_str Consejo Superior de Investigaciones Científicas (CSIC)
reponame_str DIGITAL.CSIC. Repositorio Institucional del CSIC
collection DIGITAL.CSIC. Repositorio Institucional del CSIC
repository.name.fl_str_mv
repository.mail.fl_str_mv
_version_ 1869416604845998080
score 15.198674

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