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タイトル: | Lysosomal membrane integrity in fibroblasts derived from patients with Gaucher disease |
著者: | Hamamoto, Asuka Kita, Natsuki B. Gowda, Siddabasave Gowda Takatsu, Hiroyuki Nakayama, Kazuhisa https://orcid.org/0000-0001-7701-7183 (unconfirmed) Arita, Makoto Hui, Shu-Ping Shin, Hye-Won https://orcid.org/0000-0002-9138-9554 (unconfirmed) |
著者名の別形: | 濵本, 明日香 喜多, 夏暉 髙津, 宏之 中山, 和久 申, 惠媛 |
キーワード: | glucosylceramide lysosome Gaucher disease lysosomotropic agent |
発行日: | 2024 |
出版者: | Japan Society for Cell Biology |
誌名: | Cell Structure and Function |
巻: | 49 |
号: | 1 |
開始ページ: | 1 |
終了ページ: | 10 |
抄録: | Gaucher disease (GD) is a recessively inherited lysosomal storage disorder characterized by a deficiency of lysosomal glucocerebrosidase (GBA1). This deficiency results in the accumulation of its substrate, glucosylceramide (GlcCer), within lysosomes. Here, we investigated lysosomal abnormalities in fibroblasts derived from patients with GD. It is noteworthy that the cellular distribution of lysosomes and lysosomal proteolytic activity remained largely unaffected in GD fibroblasts. However, we found that lysosomal membranes of GD fibroblasts were susceptible to damage when exposed to a lysosomotropic agent. Moreover, the susceptibility of lysosomal membranes to a lysosomotropic agent could be partly restored by exogenous expression of wild-type GBA1. Here, we report that the lysosomal membrane integrity is altered in GD fibroblasts, but lysosomal distribution and proteolytic activity is not significantly altered. |
著作権等: | © 2024 The Author(s) This is an open access article distributed under the terms of the Creative Commons BY (Attribution) License, which permits the unrestricted distribution, reproduction and use of the article provided the original source and authors are credited. |
URI: | http://hdl.handle.net/2433/287247 |
DOI(出版社版): | 10.1247/csf.23066 |
PubMed ID: | 38072450 |
出現コレクション: | 学術雑誌掲載論文等 |
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