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Reference: Luca CD, et al. (2009) Can yeast be used to study mitochondrial diseases? Biolistic tRNA mutants for the analysis of mechanisms and suppressors. Mitochondrion 9(6):408-17

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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.

Reference Type
Journal Article
Authors
Luca CD, Zhou Y, Montanari A, Morea V, Oliva R, Besagni C, Bolotin-Fukuhara M, Frontali L, Francisci S
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