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.
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”
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.
Nobuhiro Takahashi, Ph. D.,
Professor, Graduate School of Agriculture, Tokyo University of Agriculture & Technology
Life Innovation Group, Department of Innovation Research, JST