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Miyata, Takashi Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Hagiwara, Daisuke Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Hodai, Yuichi Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Miwata, Tsutomu Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Kawaguchi, Yohei Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Kurimoto, Junki Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Ozaki, Hajime Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Mitsumoto, Kazuki Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Takagi, Hiroshi Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Suga, Hidetaka Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Kobayashi, Tomoko Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Sugiyama, Mariko Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Onoue, Takeshi Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Ito, Yoshihiro Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Iwama, Shintaro Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Banno, Ryoichi Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Matsumoto, Mami Section of Electron Microscopy, Supportive Center for Brain Research, National Institute for Physiological Sciences
Kawakami, Natsuko Section of Electron Microscopy, Supportive Center for Brain Research, National Institute for Physiological Sciences
Ohno, Nobuhiko Department of Anatomy, Division of Histology and Cell Biology, Jichi Medical University, School of Medicine
Sakamoto, Hirotaka Ushimado Marine Institute, Graduate School of Natural Science and Technology, Okayama University ORCID Kaken ID publons researchmap
Arima, Hiroshi Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
Abstract
Misfolded or unfolded proteins in the ER are said to be degraded only after translocation or isolation from the ER. Here, we describe a mechanism by which mutant proteins are degraded within the ER. Aggregates of mutant arginine vasopressin (AVP) precursor were confined to ER-associated compartments (ERACs) connected to the ER in AVP neurons of a mouse model of familial neurohypophysial diabetes insipidus. The ERACs were enclosed by membranes, an ER chaperone and marker protein of phagophores and autophagosomes were expressed around the aggregates, and lysosomes fused with the ERACs. Moreover, lysosome-related molecules were present within the ERACs, and aggregate degradation within the ERACs was dependent on autophagic-lysosomal activity. Thus, we demonstrate that protein aggregates can be degraded by autophagic-lysosomal machinery within specialized compartments of the ER.
Published Date
2020-10-23
Publication Title
iScience
Volume
volume23
Issue
issue10
Publisher
Cell Press
Start Page
101648
ISSN
2589-0042
Content Type
Journal Article
language
English
OAI-PMH Set
岡山大学
Copyright Holders
© 2020 The Authors.
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isVersionOf https://doi.org/10.1016/j.isci.2020.101648
License
http://creativecommons.org/licenses/by-nc-nd/4.0/
Funder Name
Japan Society for the Promotion of Science
助成番号
JP15K19530
JP16H06280