天然免疫分子在糖基化异常IgA致肾小球足细胞损伤中的免疫调节作用
李 晓,周 同*,陈 楠
(上海交通大学医学院附属瑞金医院,上海 200025)

摘 要:

   摘 要:IgA肾病(IgA nephropathy, IgAN)位居各类肾小球疾病之首,是一组以IgA为主的免疫球蛋白在肾小球系膜区沉积为特征的免疫介导性肾小球疾病,也是引起患者终末期肾衰竭最常见的病因之一。足细胞是继系膜细胞与IgA肾病关系的新近关注热点,其系一类位于基底膜最外层的上皮细胞,并是构成肾小球滤过屏障的核心成份。目前认为,足细胞损伤及其生物学行为在IgA肾病等疾病起始进展乃至终末期肾衰中起关键作用。近年伴随着对上皮细胞尤其细胞转分化(EMT)现象在足细胞损伤机制中重要意义的认识,人们注意到糖基化异常IgA在足细胞EMT发生中的诱发作用,以及足细胞EMT过程中的病生理调控机制与IgA肾病等肾小球疾病发生发展的关系。为此,该文进一步基于足细胞的生物学特性以及免疫调节新的视角,探讨天然免疫分子在糖基化异常IgA致足细胞损伤中的调控作用,拟为进一步阐释IgA肾病发病机制及其相关研究乃至临床治疗提供新的思路。
关键词:肾小球疾病;足细胞;IgA;DC-SIGN;免疫调节
中图分类号:R692.3 文献标识码:A


    


 

The immune regulation effects of innate immune molecules on deglycosylated IgA induced glomerular podocytes injury
LI Xiao, ZHOU Tong *, CHEN Nan
(Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China)

Abstract:

Abstract: IgA nephropathy (IgAN), the most common primary glomerulonephritis worldwide, is one of the leading causes of end stage renal disease. The disease is characterized by depositions of IgA in the glomerular mesangium. Podocytes are highly differentiated cells that play a key role in maintaining the integrity of the glomerular filtration barrier. Recently, they have addressed the attention in the pathogenesis of IgA nephropathy following mesangial cells. Podocytes damage and their biological behavior may take part in the progression of renal diseases such as IgA nephropathy. Epithelial-mesenchymal transition (EMT) has been recognized to play an important role in podocyte injury, while deglycosylated IgA1 may lead to podocyte EMT. However, the pathological modular mechanism of podocyte EMT and the relationship with the pathogenesis of IgA nephropathy are to be investigated. In this review, the immune regulating effects of innate immune molecules on deglycosylated IgA induced podocytes injury are discussed to further provide new insights in the pathogenesis and therapeutics in IgA nephropathy.
Key words: glomerular diseases; podocyte; IgA; DC-SIGN; immune-regulation

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