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Kidney Diseases Caused by Complement Dysregulation: Acquired, Inherited, and Still More to Come

Inherited and acquired dysregulation of the complement alternative pathway plays an important role in multiple renal diseases. In recent years, the identification of disease-causing mutations and genetic variants in complement regulatory proteins has contributed significantly to our knowledge of the...

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Autores principales: Heeringa, Saskia F., Cohen, Clemens D.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3511829/
https://www.ncbi.nlm.nih.gov/pubmed/23227086
http://dx.doi.org/10.1155/2012/695131
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author Heeringa, Saskia F.
Cohen, Clemens D.
author_facet Heeringa, Saskia F.
Cohen, Clemens D.
author_sort Heeringa, Saskia F.
collection PubMed
description Inherited and acquired dysregulation of the complement alternative pathway plays an important role in multiple renal diseases. In recent years, the identification of disease-causing mutations and genetic variants in complement regulatory proteins has contributed significantly to our knowledge of the pathogenesis of complement associated glomerulopathies. In these diseases defective complement control leading to the deposition of activated complement products plays a key role. Consequently, complement-related glomerulopathies characterized by glomerular complement component 3 (C3) deposition in the absence of local immunoglobulin deposits are now collectively described by the term “C3 glomerulopathies.” Therapeutic strategies for reestablishing complement regulation by either complement blockade with the anti-C5 monoclonal antibody eculizumab or plasma substitution have been successful in several cases of C3 glomerulopathies. However, further elucidation of the underlying defects in the alternative complement pathway is awaited to develop pathogenesis-specific therapies.
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spelling pubmed-35118292012-12-07 Kidney Diseases Caused by Complement Dysregulation: Acquired, Inherited, and Still More to Come Heeringa, Saskia F. Cohen, Clemens D. Clin Dev Immunol Review Article Inherited and acquired dysregulation of the complement alternative pathway plays an important role in multiple renal diseases. In recent years, the identification of disease-causing mutations and genetic variants in complement regulatory proteins has contributed significantly to our knowledge of the pathogenesis of complement associated glomerulopathies. In these diseases defective complement control leading to the deposition of activated complement products plays a key role. Consequently, complement-related glomerulopathies characterized by glomerular complement component 3 (C3) deposition in the absence of local immunoglobulin deposits are now collectively described by the term “C3 glomerulopathies.” Therapeutic strategies for reestablishing complement regulation by either complement blockade with the anti-C5 monoclonal antibody eculizumab or plasma substitution have been successful in several cases of C3 glomerulopathies. However, further elucidation of the underlying defects in the alternative complement pathway is awaited to develop pathogenesis-specific therapies. Hindawi Publishing Corporation 2012 2012-11-14 /pmc/articles/PMC3511829/ /pubmed/23227086 http://dx.doi.org/10.1155/2012/695131 Text en Copyright © 2012 S. F. Heeringa and C. D. Cohen. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Article
Heeringa, Saskia F.
Cohen, Clemens D.
Kidney Diseases Caused by Complement Dysregulation: Acquired, Inherited, and Still More to Come
title Kidney Diseases Caused by Complement Dysregulation: Acquired, Inherited, and Still More to Come
title_full Kidney Diseases Caused by Complement Dysregulation: Acquired, Inherited, and Still More to Come
title_fullStr Kidney Diseases Caused by Complement Dysregulation: Acquired, Inherited, and Still More to Come
title_full_unstemmed Kidney Diseases Caused by Complement Dysregulation: Acquired, Inherited, and Still More to Come
title_short Kidney Diseases Caused by Complement Dysregulation: Acquired, Inherited, and Still More to Come
title_sort kidney diseases caused by complement dysregulation: acquired, inherited, and still more to come
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3511829/
https://www.ncbi.nlm.nih.gov/pubmed/23227086
http://dx.doi.org/10.1155/2012/695131
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