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Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family

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 co...

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Autores principales: Sánchez-Corral, Pilar, Pouw, Richard B., López-Trascasa, Margarita, Józsi, Mihály
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6052053/
https://www.ncbi.nlm.nih.gov/pubmed/30050540
http://dx.doi.org/10.3389/fimmu.2018.01607
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author Sánchez-Corral, Pilar
Pouw, Richard B.
López-Trascasa, Margarita
Józsi, Mihály
author_facet Sánchez-Corral, Pilar
Pouw, Richard B.
López-Trascasa, Margarita
Józsi, Mihály
author_sort Sánchez-Corral, Pilar
collection PubMed
description 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.
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spelling pubmed-60520532018-07-26 Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family Sánchez-Corral, Pilar Pouw, Richard B. López-Trascasa, Margarita Józsi, Mihály Front Immunol Immunology 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. Frontiers Media S.A. 2018-07-12 /pmc/articles/PMC6052053/ /pubmed/30050540 http://dx.doi.org/10.3389/fimmu.2018.01607 Text en Copyright © 2018 Sánchez-Corral, Pouw, López-Trascasa and Józsi. https://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Immunology
Sánchez-Corral, Pilar
Pouw, Richard B.
López-Trascasa, Margarita
Józsi, Mihály
Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family
title Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family
title_full Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family
title_fullStr Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family
title_full_unstemmed Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family
title_short Self-Damage Caused by Dysregulation of the Complement Alternative Pathway: Relevance of the Factor H Protein Family
title_sort self-damage caused by dysregulation of the complement alternative pathway: relevance of the factor h protein family
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6052053/
https://www.ncbi.nlm.nih.gov/pubmed/30050540
http://dx.doi.org/10.3389/fimmu.2018.01607
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