Sequestosome 1 (SQSTM1) also known as ubiquitin-binding protein p62 (p62) is a cargo protein involved in the degradation of misfolded proteins via selective autophagy. Disruption of autophagy and resulting accumulation of misfolded proteins in the endoplasmic reticulum (ER) leads to ER stress. ER stress is implicated in several neurodegenerative diseases and obesity. As knockout of p62 (p62KO) reportedly induces obesity in mice, we examined how p62 contributes to ER stress and the ensuing unfolded protein response (UPR) in hypothalamus using mouse organotypic cultures in the present study. Cultures from p62KO mice showed significantly reduced formation of LC3-GFP puncta, an index of autophagosome formation, in response to the chemical ER stressor thapsigargin compared to wild-type (WT) cultures. Hypothalamic cultures from p62KO mice exhibited higher basal expression of the UPR/ER stress markers CHOP mRNA and ATF4 mRNA than WT cultures. Thapsigargin enhanced CHOP, ATF4, and BiP mRNA as well as p-eIF2α protein expression in both WT and p62KO cultures, but all peak values were greater in p62KO cultures. A proteasome inhibitor increased p62 expression in WT cultures and upregulated the UPR/ER stress markers CHOP mRNA and ATF4 mRNA in both genotypes, but to a greater extent in p62KO cultures. Therefore, p62 deficiency disturbed autophagosome formation and enhanced both basal and chemically induced ER stress, suggesting that p62 serves to prevent ER stress in mouse hypothalamus by maintaining protein folding capacity.

译文

:Sequestosome 1(SQSTM1)也称为泛素结合蛋白p62(p62)是一种货物蛋白,通过选择性自噬作用参与错折叠蛋白的降解。自噬的中断和错误折叠的蛋白质在内质网(ER)中的积累会导致ER应激。内质网应激与几种神经退行性疾病和肥胖有关。由于据报道,敲除p62(p62KO)会诱发小鼠肥胖,因此在本研究中,我们使用小鼠器官培养物检查了p62如何导致ER应激以及下丘脑中未折叠的蛋白反应(UPR)。与野生型(WT)培养相比,来自p62KO小鼠的培养物响应化学ER应激毒胡萝卜素,显示出LC3-GFP点的形成显着减少,这是自噬体形成的指标。来自p62KO小鼠的下丘脑培养物比WT培养物具有更高的UPR / ER应激标志物CHOP mRNA和ATF4 mRNA的基础表达。 Thapsigargin在WT和p62KO培养物中均增强了CHOP,ATF4和BiP mRNA以及p-eIF2α蛋白的表达,但在p62KO培养物中所有峰值均更大。蛋白酶体抑制剂增加了WT培养物中p62的表达,并上调了两种基因型的UPR / ER应激标记CHOP mRNA和ATF4 mRNA,但在p62KO培养物中的表达更大。因此,p62缺乏会干扰自噬体的形成,并增强基础和化学诱导的ER应激,提示p62通过维持蛋白质折叠能力来预防小鼠下丘脑的ER应激。

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