儿童原发性肾病综合征与PD-1/PD-L1信号通路

Primary nephrotic syndrome in children and PD-1 / PD-L1 signaling pathway

儿童原发性肾病综合征(primary nephrotic syndrome,PNS)是指不明原因引起的肾小球滤过膜通透性增加,导致大量蛋白尿并由此引发的一系列病理生理改变,多认为与免疫系统紊乱相关.程序性死亡受体1(programmed death 1,PD-1)属于CD28/CTLA-4家族成员,与其配体即程序性死亡受体-配体1(programmed death-ligand 1,PD-L1)结合,激活PD-1/PD-L1信号通路,抑制T细胞活化、增殖、分化以及细胞因子的分泌,负性调节免疫反应,提高组织免疫耐受.以下简要介绍儿童PNS与PD-1/PD-L1信号通路的研究最新进展.

Primary nephrotic syndrome(PNS)in children is a series of physiological and pathological changes caused by unknown reasons,in which increased glomerular filtration membrane permeability results in a large amount of proteinuria. It is considered to be related with immune system disorders. Programmed death 1 (PD-1)is a member of the CD28 / CTLA-4 family. The binding of PD-1 and its ligand(programmed death ligand 1,PD-L1)can activate PD-1 / PD-L1 signaling pathway,which inhibits T cell activation,proliferation,differentia-tion and cytokine secretion,and negatively regulate immune responses and improve immune tolerance. This re-view briefly introduces the latest research progress of PNS in children and PD-1 / PD-L1 signaling pathway.