| Literature DB >> 19631764 |
Cristina De Luca1, YouFang Zhou, Arianna Montanari, Veronica Morea, Romina Oliva, Celine Besagni, Monique Bolotin-Fukuhara, Laura Frontali, Silvia Francisci.
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 synthetase. The effect of suppressors was analyzed 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.Entities:
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Year: 2009 PMID： 19631764 DOI： 10.1016/j.mito.2009.07.004
Source DB: PubMed Journal: Mitochondrion ISSN： 1567-7249 Impact factor: 4.160