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Publication : Endoplasmic reticulum-associated degradation regulates mitochondrial dynamics in brown adipocytes.

First Author  Zhou Z Year  2020
Journal  Science Volume  368
Issue  6486 Pages  54-60
PubMed ID  32193362 Mgi Jnum  J:286589
Mgi Id  MGI:6402841 Doi  10.1126/science.aay2494
Citation  Zhou Z, et al. (2020) Endoplasmic reticulum-associated degradation regulates mitochondrial dynamics in brown adipocytes. Science 368(6486):54-60
abstractText  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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