dc.contributor.author | Sánchez-Corral, Pilar | |
dc.contributor.author | Pouw, Richard B. | |
dc.contributor.author | López Trascasa, Margarita | |
dc.contributor.author | Józsi, Mihály | |
dc.contributor.other | UAM. Departamento de Medicina | es_ES |
dc.contributor.other | Instituto de Investigación Sanitaria Hospital Universitario de La Paz (IdiPAZ) | es_ES |
dc.date.accessioned | 2018-09-26T13:42:58Z | |
dc.date.available | 2018-09-26T13:42:58Z | |
dc.date.issued | 2018-07-12 | |
dc.identifier.citation | Frontiers in Immunology 9.July (2018): Article 1607 | en_US |
dc.identifier.issn | 664-322 | es_ES |
dc.identifier.uri | http://hdl.handle.net/10486/685193 | |
dc.description.abstract | The alternative pathway is a continuously active surveillance arm of the complement system, and it can also enhance complement activation initiated by the classical and the lectin pathways. Various membrane-bound and plasma regulatory proteins control the activation of the potentially deleterious complement system. Among the regulators, the plasma glycoprotein factor H (FH) is the main inhibitor of the alternative pathway and its powerful amplification loop. FH belongs to a protein family that also includes FH-like protein 1 and five factor H-related (FHR-1 to FHR-5) proteins. Genetic variants and abnormal rearrangements involving the FH protein family have been linked to numerous systemic and organ-specific diseases, including age-related macular degeneration, and the renal pathologies atypical hemolytic uremic syndrome, C3 glomerulopathies, and IgA nephropathy. This review covers the known and recently emerged ligands and interactions of the human FH family proteins associated with disease and discuss the very recent experimental data that suggest FH-antagonistic and complement-activating functions for the FHR proteins | en_US |
dc.description.sponsorship | PS-C and ML-T are funded by grants PI16/00723 and PI15/00255 (Spanish Ministerio de Economía y Competitividad/ISCIII, and European Program FEDER) and B2017/BMD3673 (Complement II-CM network from the Comunidad de Madrid). MJ is supported by the National Research, Development and Innovation Fund of Hungary (NKFIA grants K 109055 and K 125219), the Kidneeds Foundation, Iowa, US, and by the Institutional
Excellence Program of the Ministry of Human Capacities of Hungary | en_US |
dc.format.extent | 19 pag. | es_ES |
dc.format.mimetype | application/pdf | en |
dc.language.iso | eng | en |
dc.publisher | Frontiers Media | en_US |
dc.relation.ispartof | Frontiers in Immunology | en_US |
dc.rights | © 2018 Sánchez-Corral, Pouw, López-Trascasa and Józsi. | es_ES |
dc.subject.other | Age-related macular degeneration | en_US |
dc.subject.other | Atypical hemolytic uremic syndrome | en_US |
dc.subject.other | C3 glomerulopathy | en_US |
dc.subject.other | Complement activation | en_US |
dc.subject.other | Complement de-regulation | en_US |
dc.subject.other | Factor H | en_US |
dc.subject.other | Factor H-related protein | en_US |
dc.subject.other | Opsonization | en_US |
dc.title | Self-damage caused by dysregulation of the complement alternative pathway: Relevance of the factor H protein family | en_US |
dc.type | article | en |
dc.subject.eciencia | Medicina | es_ES |
dc.relation.publisherversion | https://doi.org/10.3389/fimmu.2018.01607 | es_ES |
dc.identifier.doi | 10.3389/fimmu.2018.01607 | es_ES |
dc.identifier.publicationfirstpage | 1607-1 | es_ES |
dc.identifier.publicationissue | July | es_ES |
dc.identifier.publicationlastpage | 1607-19 | es_ES |
dc.identifier.publicationvolume | 9 | es_ES |
dc.relation.projectID | Gobierno de España. PI16/00723 | es_ES |
dc.relation.projectID | Gobierno de España. PI15/00255 | es_ES |
dc.relation.projectID | Comunidad de Madrid. B2017/BMD-3673/COMPLEMENTOII-CM//IMAS12 | es_ES |
dc.type.version | info:eu-repo/semantics/publishedVersion | en |
dc.rights.cc | Reconocimiento | es_ES |
dc.rights.accessRights | openAccess | en |
dc.facultadUAM | Facultad de Medicina | |
dc.institutoUAM | Instituto de Investigación Sanitaria Hospital Universitario de La Paz (IdiPAZ) | |