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Complement inhibition in C3 glomerulopathy
DOI:10.1016/j.smim.2016.06.002.png)
摘要
En 中文
C3 glomerulopathy (C3G) describes a spectrum of glomerular diseases defined by shared renal biopsy pathology: a predominance of C3 deposition on immunofluorescence with electron microscopy permitting disease sub-classification. Complement dysregulation underlies the observed pathology, a causal relationship that is supported by well described studies of genetic and acquired drivers of disease. In this article, we provide an overview of the features of C3G, including a discussion of disease definition and a review of the causal role of complement. We discuss molecular markers of disease and how biomarkers are informing our evolving understanding of underlying pathology. Research advances are laying the foundation for complement inhibition as a targeted approach to treatment of C3G. (C) 2016 Elsevier Ltd. All rights reserved.
Keyword:
Membranoproliferative glomerulonephritis
C3 glomerulopathy
Complement
Dense deposit disease
C3 glomerulonephritis
Eculizumab
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机构
引用论文
In situ complement activation in porcine membranoproliferative glomerulonephritis type II
KIDNEY INTERNATIONAL
IF12.6
Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor H
NATURE GENETICS
IF31.8

