カワジリ　スミヒロ   KAWAJIRI Sumihiro   河尻　澄宏    所属   医学部 医学科（附属東洋医学研究所）    職種   准教授
論文種別	原著
言語種別	英語
査読の有無	査読あり
表題	Mutations in Fis1 disrupt orderly disposal of defective mitochondria.
掲載誌名	正式名：Molecular biology of the cell 略　 称：Mol Biol Cell ISSNコード：19394586/10591524
掲載区分	国外
巻・号・頁	25(1),pp.145-59
著者・共著者	Shen Qinfang, Yamano Koji, Head Brian P, Kawajiri Sumihiro, Cheung Jesmine T M, Wang Chunxin, Cho Jeong-Hoon, Hattori Nobutaka, Youle Richard J, van der Bliek Alexander M
発行年月	2014/01
概要	Mitochondrial fission is mediated by the dynamin-related protein Drp1 in metazoans. Drp1 is recruited from the cytosol to mitochondria by the mitochondrial outer membrane protein Mff. A second mitochondrial outer membrane protein, named Fis1, was previously proposed as recruitment factor, but Fis1(-/-) cells have mild or no mitochondrial fission defects. Here we show that Fis1 is nevertheless part of the mitochondrial fission complex in metazoan cells. During the fission cycle, Drp1 first binds to Mff on the surface of mitochondria, followed by entry into a complex that includes Fis1 and endoplasmic reticulum (ER) proteins at the ER-mitochondrial interface. Mutations in Fis1 do not normally affect fission, but they can disrupt downstream degradation events when specific mitochondrial toxins are used to induce fission. The disruptions caused by mutations in Fis1 lead to an accumulation of large LC3 aggregates. We conclude that Fis1 can act in sequence with Mff at the ER-mitochondrial interface to couple stress-induced mitochondrial fission with downstream degradation processes.
DOI	10.1091/mbc.E13-09-0525
PMID	24196833