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Title: UPR transducer BBF2H7 allows export of type II collagen in a cargo- and developmental stage–specific manner
Authors: Ishikawa, Tokiro  kyouindb  KAKEN_id  orcid (unconfirmed)
Toyama, Takuya
Nakamura, Yuki
Tamada, Kentaro
Shimizu, Hitomi
Ninagawa, Satoshi  kyouindb  KAKEN_id
Okada, Tetsuya  kyouindb  KAKEN_id
Kamei, Yasuhiro
Ishikawa-Fujiwara, Tomoko
Todo, Takeshi
Aoyama, Eriko
Takigawa, Masaharu
Harada, Akihiro
Mori, Kazutoshi  kyouindb  KAKEN_id
Author's alias: 石川, 時郎
遠山, 拓也
中村, 有貴
玉田, 健太郎
清水, 瞳
蜷川, 暁
岡田, 徹也
森, 和俊
Keywords: Development
Protein Homeostasis
Issue Date: 5-Jun-2017
Publisher: Rockefeller University Press
Journal title: The Journal of Cell Biology
Volume: 216
Issue: 6
Start page: 1761
End page: 1774
Abstract: The unfolded protein response (UPR) handles unfolded/misfolded proteins accumulated in the endoplasmic reticulum (ER). However, it is unclear how vertebrates correctly use the total of ten UPR transducers. We have found that ER stress occurs physiologically during early embryonic development in medaka fish and that the smooth alignment of notochord cells requires ATF6 as a UPR transducer, which induces ER chaperones for folding of type VIII (short-chain) collagen. After secretion of hedgehog for tissue patterning, notochord cells differentiate into sheath cells, which synthesize type II collagen. In this study, we show that this vacuolization step requires both ATF6 and BBF2H7 as UPR transducers and that BBF2H7 regulates a complete set of genes (Sec23/24/13/31, Tango1, Sedlin, and KLHL12) essential for the enlargement of COPII vesicles to accommodate long-chain collagen for export, leading to the formation of the perinotochordal basement membrane. Thus, the most appropriate UPR transducer is activated to cope with the differing physiological ER stresses of different content types depending on developmental stage.
Rights: © 2017 Ishikawa et al. This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 4.0 International license, as described at
DOI(Published Version): 10.1083/jcb.201609100
PubMed ID: 28500182
Appears in Collections:Journal Articles

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