Alport syndrome is a hereditary kidney disease caused by collagen IV mutations that interfere with the formation and deposition of the α3α4α5 protomer into the glomerular basement membrane. In this issue, Yu et al. show that the chemical chaperone tauroursodeoxycholic acid prevented kidney structural changes and function decline in mice with a pathogenic missense Col4a3 mutation by increasing mutant α3α4α5 protomer glomerular basement membrane deposition and preventing podocyte apoptosis induced by endoplasmic reticulum stress.

中文翻译：

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化学伴侣可以拯救阿尔波特综合征？


Alport 综合征是一种由 IV 型胶原蛋白突变引起的遗传性肾脏疾病，该突变干扰 α3α4α5 原聚体的形成和沉积到肾小球基底膜中。在本期中，Yu 等人。结果表明，化学伴侣牛磺熊去氧胆酸通过增加突变体α3α4α5原体肾小球基底膜沉积并防止内质网应激诱导的足细胞凋亡，防止具有致病性错义Col4a3突变的小鼠的肾脏结构变化和功能下降。