IgA 肾病的遗传学和免疫生物学。
The genetics and immunobiology of IgA nephropathy.
出版信息
J Clin Invest. 2014 Jun;124(6):2325-32. doi: 10.1172/JCI74475. Epub 2014 Jun 2.
Abstract
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.
摘要
IgA 肾病 (IgAN) 是东亚人群肾衰竭的主要病因，也是欧洲人群原发性肾小球肾炎最常见的形式。IgAN 患者会产生含有特征性 IgA1 的免疫复合物，这些复合物会在肾小球系膜中沉积，导致进行性的肾脏损伤。最近的研究将 IgAN 定义为一种具有复杂结构和强烈遗传决定因素的自身免疫特征。本综述总结了在 IgAN 发病机制中，O-糖基化途径、抗糖免疫反应、黏膜免疫、抗原加工和呈递以及替代补体途径的新见解。
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