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TDP-43 stabilises the processing intermediates of mitochondrial transcripts

TDP-43 stabilises the processing intermediates of mitochondrial transcripts

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  • 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
  • Nobuhiro Takahashi (Lead / Corresponding author)

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Original languageEnglish
Article number7709
Number of pages14
JournalScientific Reports
StatePublished - 9 Aug 2017


The 43-kDa trans-activating response region DNA-binding protein 43 (TDP-43) is a product of a causative gene for amyotrophic lateral sclerosis (ALS). Despite of accumulating evidence that mitochondrial dysfunction underlies the pathogenesis of TDP-43-related ALS, the roles of wild-type TDP-43 in mitochondria are unknown. Here, we show that the small TDP-43 population present in mitochondria binds directly to a subset of mitochondrial tRNAs and precursor RNA encoded in L-strand mtDNA. Upregulated expression of TDP-43 stabilised the processing intermediates of mitochondrial polycistronic transcripts and their products including the components of electron transport and 16S mt-rRNA, similar to the phenotype observed in cells deficient for mitochondrial RNase P. Conversely, TDP-43 deficiency reduced the population of processing intermediates and impaired mitochondrial function. We propose that TDP-43 has a novel role in maintaining mitochondrial homeostasis by regulating the processing of mitochondrial transcripts.

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