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JST Press Release

August 9, 2017
Japan Science and Technology Agency (JST)
5-3, Yonbancho, Chiyoda-ku, Tokyo 102-8666

Uncovered new mitochondrial function of TDP-43, a causative gene product of amyotrophic lateral sclerosis

Expectations for development of therapeutic drugs such as refractory cerebral neurodegenerative diseases

Unanticipated roles of TDP-43 that provide new insight into the pathogenesis of ALS are elucidated. TDP-43 is a product of the causative gene of familial and sporadic ALS and found accumulated in the damaged motor neurons of ~90% ALS patients. This study revealed novel roles of TDP-43 in mitochondria; it regulates the levels of particular tRNAs/mRNAs in mitochondria by stabilizing the processing intermediates of mitochondrial DNA transcripts. This finding will lead to the development of novel targets for early diagnosis and treatment of this severe neurodegenerative disease.

Program Information

Research Area “Structural Life Science and Advanced Core Technologies for Innovative Life Science Research”
Research Theme “Molecular studies of RNA-dysmetabolic syndrome –From ribonucleoproteomics to structural life science”

Journal Information

Keiichi Izumikawa, Yuko Nobe, Harunori Yoshikawa, Hideaki Ishikawa, Yutaka Miura, Hiroshi Nakayama, Takashi Nonaka, Masato Hasegawa, Naohiro Egawa, Haruhisa Inoue, Kouki Nishikawa, Koji Yamano, Richard J Simpson, Masato Taoka, Yoshio Yamauchi, Toshiaki Isobe and Nobuhiro Takahashi. “TDP-43 stabilises the processing intermediates of mitochondrial transcripts”. Scientific Reports, Published online Aug. 9, 2017, doi: 10.1038/s41598-017-06953-y.


[About Research]
Nobuhiro Takahashi, Ph. D.,
Professor, Graduate School of Agriculture, Tokyo University of Agriculture & Technology

[About Program]
Tetsu Kawaguchi
Life Innovation Group, Department of Innovation Research, JST


JST, an integrated organization of science and technology in Japan, establishes an infrastructure for the entire process from the creation of knowledge to the return to the society. For more information, visit http://www.jst.go.jp/EN/