Department of Nephrology, Graduate School of Medicine, Chiba University , Chiba , Japan.
Department of Pharmacology and Therapeutics, Faculty of Medicine, Universitas Airlangga , Surabaya , Indonesia.
Am J Physiol Renal Physiol. 2018 Nov 1;315(5):F1336-F1344. doi: 10.1152/ajprenal.00292.2018. Epub 2018 Aug 15.
In many cells and tissues, including the glomerular filtration barrier, scaffold proteins are critical in optimizing signal transduction by enhancing structural stability and functionality of their ligands. Recently, mutations in scaffold protein membrane-associated guanylate kinase inverted 2 (MAGI-2) encoding gene were identified among the etiology of steroid-resistant nephrotic syndrome. MAGI-2 interacts with core proteins of multiple pathways, such as transforming growth factor-β signaling, planar cell polarity pathway, and Wnt/β-catenin signaling in podocyte and slit diaphragm. Through the interaction with its ligand, MAGI-2 modulates the regulation of apoptosis, cytoskeletal reorganization, and glomerular development. This review aims to summarize recent findings on the role of MAGI-2 and some other scaffold proteins, such as nephrin and synaptopodin, in the underlying mechanisms of glomerulopathy.
在许多细胞和组织中,包括肾小球滤过屏障,支架蛋白在通过增强其配体的结构稳定性和功能来优化信号转导方面起着至关重要的作用。最近,在类固醇抵抗性肾病综合征的病因中发现了支架蛋白膜相关鸟苷酸激酶倒置 2(MAGI-2)编码基因的突变。MAGI-2 与多种途径的核心蛋白相互作用,如足细胞和裂孔隔膜中的转化生长因子-β信号通路、平面细胞极性通路和 Wnt/β-连环蛋白信号通路。通过与配体的相互作用,MAGI-2 调节细胞凋亡、细胞骨架重排和肾小球发育的调节。本综述旨在总结 MAGI-2 及其他一些支架蛋白(如nephrin 和 synaptopodin)在肾小球病发病机制中的作用的最新发现。