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IgA肾病中IgA1异常糖基化的致病机制
引用本文:谢林伸,王立,黄俊,樊均明. IgA肾病中IgA1异常糖基化的致病机制[J]. 生物医学工程学杂志, 2010, 0(1)
作者姓名:谢林伸  王立  黄俊  樊均明
作者单位:四川大学华西医院肾内科;四川大学华西医院中央运输科;
摘    要:IgA肾病是最常见的原发性肾小球疾病,是发展为终末期肾病的主要病因,其病理机制复杂,临床表现多样化,组织形态学改变轻重不一。IgA肾病以肾小球系膜区IgA1沉积为病理特征,肾脏沉积的IgA1分子铰链区O-糖链半乳糖基减少,致使IgA1分子易于自身聚集并沉积在肾小球。糖基化酶缺乏、基因突变、免疫紊乱都可能导致IgA1异常糖基化的发生。IgA1分子的异常糖基化是IgA肾病发病的关键因素,但其具体产生原因和致病机制仍未明确,对IgA1异常糖基化的深入研究有助于了解IgA肾病的发病机制并提供新的治疗方向。

关 键 词:IgA肾病  IgA1  异常糖基化  

IgA1 Aberrant Glycosylation in the Pathogenesis of IgA Nephropathy:An Overivew
Xie Linshen Wang Li Huang Jun Fan Junming. IgA1 Aberrant Glycosylation in the Pathogenesis of IgA Nephropathy:An Overivew[J]. Journal of biomedical engineering, 2010, 0(1)
Authors:Xie Linshen Wang Li Huang Jun Fan Junming
Affiliation:Xie Linshen1 Wang Li2 Huang Jun1 Fan Junming11(Department of Nephrology,West China Hospital,Sichuan University,Chengdu 610041,China)2(Department of Central Transportation,China)
Abstract: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.Abno...
Keywords:IgA nephropathy  IgA1  Aberrant glycosylation  
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