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Can yeast be used to study mitochondrial diseases? biolistic tRNA mutants for the analysis of mechanisms and suppressors.

Abstract : Base substitutions equivalent to those causing human pathologies have been introduced in yeast mitochondrial tRNA genes. These mutants can be utilized as flexible tools to investigate the molecular aspects of mitochondrial diseases and identify correcting genes. We show that for all studied tRNA mutations (including an homoplasmic one in tRNA(Val)) the severity of phenotypes follows the same trend in four different nuclear backgrounds. Correcting genes include TUF1 and genes encoding aminoacyl-tRNA synthetases. The effect of suppressors was analysed by Northern blot. Mutated leucyl-tRNA synthetase with highly reduced catalytic activity maintains full suppressing effect, thus suggesting a chaperone-like and/or stabilizing function.
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https://hal.archives-ouvertes.fr/hal-00408939
Contributor : Marie-Claude Serre <>
Submitted on : Tuesday, August 4, 2009 - 3:01:32 PM
Last modification on : Wednesday, October 14, 2020 - 3:45:38 AM

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Cristina De Luca, Youfang Zhou, Arianna Montanari, Veronica Morea, Romina Oliva, et al.. Can yeast be used to study mitochondrial diseases? biolistic tRNA mutants for the analysis of mechanisms and suppressors.. Mitochondrion, Elsevier, 2009, epub ahead of print. ⟨10.1016/j.mito.2009.07.004⟩. ⟨hal-00408939⟩

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