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- W2116085004 abstract "'C3 glomerulopathy' is a recent disease classification comprising several rare types of glomerulonephritis (GN), including dense deposit disease (DDD), C3 glomerulonephritis (C3GN) and CFHR5 nephropathy. These disorders share the key histological feature of isolated complement C3 deposits in the glomerulus. A common aetiology involving dysregulation of the alternative pathway (AP) of complement has been elucidated in the past decade, with genetic defects and/or autoantibodies able to be identified in a proportion of patients. We review the clinical and histological features of C3 glomerulopathy, relating these to underlying molecular mechanisms. The role of uncontrolled C3 activation in pathogenesis is emphasized, with important lessons from animal models. Methods, advantages and limitations of gene testing in the assessment of individuals or families with C3 glomerulopathy are discussed. While no therapy has yet been shown consistently effective, clinical evaluation of agents targeting specific components of the complement system is ongoing. However, limits to current knowledge regarding the natural history and the appropriate timing and duration of proposed therapies need to be addressed." @default.
- W2116085004 created "2016-06-24" @default.
- W2116085004 creator A5027579674 @default.
- W2116085004 creator A5045006394 @default.
- W2116085004 creator A5073597813 @default.
- W2116085004 date "2013-03-10" @default.
- W2116085004 modified "2023-10-13" @default.
- W2116085004 title "Recent insights into C3 glomerulopathy" @default.
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- W2116085004 doi "https://doi.org/10.1093/ndt/gfs430" @default.
- W2116085004 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3707523" @default.
- W2116085004 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/23479095" @default.
- W2116085004 hasPublicationYear "2013" @default.
- W2116085004 type Work @default.