Xie Linshen, Wang Li, Huang Jan, Fan Junming
Department of Nephrology, West China Hospital, Sichuan University, Chengdu 610041, China.
Sheng Wu Yi Xue Gong Cheng Xue Za Zhi. 2010 Feb;27(1):227-30.
IgA nephropathy is the most common form of primary glomerulonephritis which mainly accounts for the development of end-stage renal diseases. It is characterized by deposits of IgA1 in mesangium. The pathogenesis of IgA nephropathy is complicated. Moreover, there is a wide range of clinical features and variable histomorphologies in the diagnosed cases of IgA nephropathy. It was demonstrated that the galactose-deficient of IgA1 O-glycan chains led IgA1 to self-aggregation and eventual deposition in mesangium. Abnormality of glycosyltransferases, genetic mutation and immunologic disorder were involved in the aberrant glycosylation of IgA1 which was recognized as the key etiopathogenisis of IgA nephropathy. However, the exact source and the pathogenic mechanism of aberrantly glycosylated IgA1 remain obscure. The further studies on aberrant O-glycosylation of IgA1 would contribute to the understanding of IgA nephropathy and provide new therapeutical strategy.
IgA肾病是原发性肾小球肾炎最常见的形式，主要导致终末期肾病的发生。其特征是IgA1在系膜中沉积。IgA肾病的发病机制复杂。此外，IgA肾病确诊病例的临床特征广泛，组织形态各异。研究表明，IgA1 O-聚糖链的半乳糖缺乏导致IgA1自我聚集并最终沉积于系膜。糖基转移酶异常、基因突变和免疫紊乱参与了IgA1的异常糖基化，这被认为是IgA肾病的关键发病机制。然而，异常糖基化IgA1的确切来源和致病机制仍不清楚。对IgA1异常O-糖基化的进一步研究将有助于理解IgA肾病并提供新的治疗策略。
