The genetics and immunobiology of IgA nephropathy

被引:230
作者
Kiryluk, Krzysztof [1 ]
Novak, Jan [2 ]
机构
[1] Columbia Univ, Dept Med, Coll Phys & Surg, New York, NY 10032 USA
[2] Univ Alabama Birmingham, Dept Microbiol, Birmingham, AL 35294 USA
关键词
GENOME-WIDE ASSOCIATION; GALACTOSE-DEFICIENT IGA1; CIRCULATING IMMUNE-COMPLEXES; COMPLEMENT FACTOR-H; HUMAN SERUM IGA1; LEUKEMIA INHIBITORY FACTOR; O-LINKED OLIGOSACCHARIDES; HINGE REGION; MASS-SPECTROMETRY; MESANGIAL IGA;
D O I
10.1172/JCI74475
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
IgA nephropathy (IgAN) represents the leading cause of kidney failure among East Asian populations and the most frequent form of primary glomerulonephritis among Europeans. Patients with IgAN develop characteristic IgA1-containing immune complexes that deposit in the glomerular mesangium, producing progressive kidney injury. Recent studies define IgAN as an autoimmune trait of complex architecture with a strong genetic determination. This Review summarizes new insights into the role of the O-glycosylation pathway, anti-glycan immune response, mucosal immunity, antigen processing and presentation, and the alternative complement pathway in the pathogenesis of IgAN.
引用
收藏
页码:2325 / 2332
页数:8
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