Ontology highlight
ABSTRACT:
SUBMITTER: Izumikawa K
PROVIDER: S-EPMC5550480 | biostudies-literature | 2017 Aug
REPOSITORIES: biostudies-literature
Scientific reports 20170809 1
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 ...[more]