内质网相关蛋白降解
内质网
细胞生物学
线粒体
脂肪生成
未折叠蛋白反应
蛋白质降解
生物
化学
间充质干细胞
作者
Zhangsen Zhou,Mauricio Torres,Haibo Sha,Christopher J. Halbrook,Françoise Van den Bergh,Rachel B. Reinert,Tatsuya Yamada,Siwen Wang,Yingying Luo,Allen H. Hunter,Chunqing Wang,Thomas H. Sanderson,Meilian Liu,Alasdair Taylor,Hiromi Sesaki,Costas A. Lyssiotis,Jun Wu,Sander Kersten,Daniel A. Beard,Ling Qi
出处
期刊:Science
[American Association for the Advancement of Science (AAAS)]
日期:2020-04-03
卷期号:368 (6486): 54-60
被引量:114
标识
DOI:10.1126/science.aay2494
摘要
The endoplasmic reticulum (ER) engages mitochondria at specialized ER domains known as mitochondria-associated membranes (MAMs). Here, we used three-dimensional high-resolution imaging to investigate the formation of pleomorphic "megamitochondria" with altered MAMs in brown adipocytes lacking the Sel1L-Hrd1 protein complex of ER-associated protein degradation (ERAD). Mice with ERAD deficiency in brown adipocytes were cold sensitive and exhibited mitochondrial dysfunction. ERAD deficiency affected ER-mitochondria contacts and mitochondrial dynamics, at least in part, by regulating the turnover of the MAM protein, sigma receptor 1 (SigmaR1). Thus, our study provides molecular insights into ER-mitochondrial cross-talk and expands our understanding of the physiological importance of Sel1L-Hrd1 ERAD.
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