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Complement inhibition in C3 glomerulopathy

delete2016-06-01
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PRE
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C
Carla Nester
R
Richard J. Smith *
DOI:10.1016/j.smim.2016.06.002delete
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摘要

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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期刊

Seminars in Immunology 封面图
Seminars in Immunology
IF:
7.8
论文数:
1.6K
被引数:
7.3K

机构

U
University of Iowa
学者数:
2.8W
论文数: 2.3W
被引数: 600
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