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論文
- タイトル
- タイトル（英）
- Discovery of a protein uptake pathway in lysosomes
- 参照URL
- https://researchmap.jp/koronku/published_papers/40185708
- 著者
- 著者（英）
- Yuuki Fujiwara,Viorica Raluca Contu,Chihana Kabuta,Megumu Ogawa,Hiromi Fujita,Hisae Kikuchi,Ryohei Sakai,Katsunori Hase,Mari Suzuki,Ikuko Koyama-Honda,Michio Inoue,Yasushi Oya,Yukiko U. Inoue,Takayoshi Inoue,Ryosuke Takahashi,Ichizo Nishino,Keiji Wada,Satoru Noguchi,Tomohiro Kabuta
- 担当区分
- 概要
- 概要（英）
- Abstract The degradation of cellular components plays an essential role in homeostasis. However, the known degradation pathways cannot account for the levels of proteolysis in cells. Here, we demonstrate that cytosolic proteins are imported into lysosomes in an ATP-dependent manner for degradation through a direct uptake mechanism distinct from any known pathway. SIDT2, a lysosomal membrane protein previously reported as an RNA transporter, translocates substrate proteins across the lysosomal membrane. Furthermore, we identify a dominant-negative mutation in SIDT2 that causes neuropathy and distal myopathy with rimmed vacuoles, a protein aggregation disease in humans. We generate Sidt2 knockout mice, recapitulating the characteristic features of this disease. Our results reveal a novel degradation pathway and illustrate its crucial role in cellular proteostasis, physiology, and pathophysiology. One Sentence Summary Discovery of a novel proteolytic pathway in cells, the dysfunction of which leads to protein aggregation disease in humans.
- 出版者・発行元
- 出版者・発行元（英）
- Cold Spring Harbor Laboratory
- 誌名
- 誌名（英）
- bioRxiv
- 巻
- 号
- 開始ページ
- 2020.08.11.245688
- 終了ページ
- 出版年月
- 2020年8月11日
- 査読の有無
- 招待の有無
- 掲載種別
- ISSN
- DOI URL
- https://doi.org/10.1101/2020.08.11.245688
- 共同研究・競争的資金等の研究課題
研究者
鈴木 マリ
(スズキ マリ)