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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...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi Publishing Corporation
2012
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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. |
format | Online Article Text |
id | pubmed-3511829 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2012 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
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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