Here we demonstrate association of variants in the mitochondrial asparaginyl-tRNA
synthetase NARS2 with human hearing loss and Leigh syndrome. A homozygous missense …

Translation of ribosomal protein-coding mRNAs (RP-mRNAs) constitutes a key step in
ribosome biogenesis, but the mechanisms that modulate RP-mRNA translation in coordination …

Purpose: Nucleoside analogues form the backbone of many therapeutic regimens in
oncology and require the presence of intracellular enzymes for their activation. A ProTide is …

The canine transmissible venereal tumor (CTVT) is a clonally transmissible cancer that
regresses spontaneously or after treatment with vincristine, but we know little about the …

Mitochondria are considered as the powerhouse of eukaryotic cells. They host several
central metabolic processes fueling the oxidative phosphorylation pathway (OXPHOS) that …

In eukaryotes, tRNAs are transcribed in the nucleus and exported to the cytosol, where they
deliver amino acids to ribosomes for protein translation. This nuclear-cytoplasmic movement …

Mutations in human mitochondrial aminoacyl-tRNA synthetases are associated with a
variety of neurodegenerative disorders. The effects of these mutations on the structure and …

Human mitochondrial aminoacyl-tRNA synthetases (mt-aaRSs) are encoded in the nucleus,
synthesized in the cytosol and targeted for importation into mitochondria by a N-terminal …

LARP1 is an oncogenic RNA-binding protein required for ribosome biogenesis and cancer
cell survival. From published in vitro studies, there is disparity over which of two different …

Mutations in the nuclear gene coding for the mitochondrial aspartyl-tRNA synthetase, a key
enzyme for mitochondrial translation, are correlated with leukoencephalopathy. Ser45 to …