ID | 61154 |
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Author |
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
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Arima, Hiroshi
Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine
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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.
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Published Date | 2020-10-23
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Publication Title |
iScience
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Volume | volume23
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Issue | issue10
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Publisher | Cell Press
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Start Page | 101648
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ISSN | 2589-0042
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Content Type |
Journal Article
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language |
English
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OAI-PMH Set |
岡山大学
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Copyright Holders | © 2020 The Authors.
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File Version | publisher
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PubMed ID | |
DOI | |
Web of Science KeyUT | |
Related Url | isVersionOf https://doi.org/10.1016/j.isci.2020.101648
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License | http://creativecommons.org/licenses/by-nc-nd/4.0/
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Funder Name |
Japan Society for the Promotion of Science
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助成番号 | JP15K19530
JP16H06280
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