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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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | Clinical and Developmental Immunology |
| Online Access: | http://dx.doi.org/10.1155/2012/695131 |
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| author | Saskia F. Heeringa Clemens D. Cohen |
| author_facet | Saskia F. Heeringa Clemens D. Cohen |
| author_sort | Saskia F. Heeringa |
| collection | DOAJ |
| 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 | Article |
| id | doaj-art-ed8d4977539d46628292d0ff1e20c5c8 |
| institution | DOAJ |
| issn | 1740-2522 1740-2530 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Clinical and Developmental Immunology |
| spelling | doaj-art-ed8d4977539d46628292d0ff1e20c5c82025-08-20T03:21:12ZengWileyClinical and Developmental Immunology1740-25221740-25302012-01-01201210.1155/2012/695131695131Kidney Diseases Caused by Complement Dysregulation: Acquired, Inherited, and Still More to ComeSaskia F. Heeringa0Clemens D. Cohen1Division of Internal Medicine, University Hospital Zurich, Raemistrasse 100, 8006 Zurich, SwitzerlandDivision of Nephrology, University Hospital Zurich, Raemistrasse 100, 8006 Zurich, SwitzerlandInherited 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.http://dx.doi.org/10.1155/2012/695131 |
| spellingShingle | Saskia F. Heeringa Clemens D. Cohen Kidney Diseases Caused by Complement Dysregulation: Acquired, Inherited, and Still More to Come Clinical and Developmental Immunology |
| 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 |
| url | http://dx.doi.org/10.1155/2012/695131 |
| work_keys_str_mv | AT saskiafheeringa kidneydiseasescausedbycomplementdysregulationacquiredinheritedandstillmoretocome AT clemensdcohen kidneydiseasescausedbycomplementdysregulationacquiredinheritedandstillmoretocome |