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Literature summary for 6.1.1.4 extracted from

  • Park, H.; Davidson, E.; King, M.P.
    Overexpressed mitochondrial leucyl-tRNA synthetase suppresses the A3243G mutation in the mitochondrial tRNA(Leu(UUR)) gene (2008), RNA, 14, 2407-2416.
    View publication on PubMedView publication on EuropePMC

Application

Application Comment Organism
medicine suppression of a tRNA gene mutation by increasing the steady-state levels of its cognate aminoacyl-tRNA synthetase is a model for potential therapies for human pathogenic tRNA mutations Homo sapiens

Protein Variants

Protein Variants Comment Organism
A3243G respiratory chain defects in A3243G mutant cells is suppressed by overexpressing human mitochondrial leucyl-tRNA synthetase. The rates of oxygen consumption in suppressed cells are directly proportional to the levels of leucyl-tRNA synthetase. 15fold higher levels of leucyl-tRNA synthetase results in wild-type respiratory chain function. The suppressed cells have increased steady-state levels of tRNA(Leu(UUR)) and up to 3fold higher steady-state levels of mitochondrial translation products, but do not have rates of protein synthesis above those in parental mutant cells Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
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Synonyms

Synonyms Comment Organism
Leucyl-tRNA synthetase
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Homo sapiens