Can yeast be used to study mitochondrial diseases? Biolistic tRNA mutants for the analysis of mechanisms and suppressors (Articolo in rivista)

Type
Label
  • Can yeast be used to study mitochondrial diseases? Biolistic tRNA mutants for the analysis of mechanisms and suppressors (Articolo in rivista) (literal)
Anno
  • 2009-01-01T00:00:00+01:00 (literal)
Alternative label
  • De Luca C; Zhou Y; Montanari A; Morea V; Oliva R; Besagni C; Bolotin-Fukuhara M; Frontali L; Francisci S. (2009)
    Can yeast be used to study mitochondrial diseases? Biolistic tRNA mutants for the analysis of mechanisms and suppressors
    in Mitochondrion (Amsterdam. Print)
    (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
  • De Luca C; Zhou Y; Montanari A; Morea V; Oliva R; Besagni C; Bolotin-Fukuhara M; Frontali L; Francisci S. (literal)
Pagina inizio
  • 408 (literal)
Pagina fine
  • 417 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume
  • 9 (literal)
Rivista
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  • PubMe (literal)
  • ISI Web of Science (WOS) (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
  • Department of Cell and Developmental Biology, Pasteur Institute-Fondazione Cenci Bolognetti, University Sapienza, 00185 Rome, Italy Laboratoire de Genetique Moleculaire, Institut de Genetique et Microbiologie UMR CNRS 8621, Universite Paris Sud, 91405 Orsay Cedex, France CNR Institute of Molecular Biology and Pathology (IBPM), c/o Department of Biochemical Sciences, University Sapienza, 00185 Rome, Italy Department of Applied Sciences, University Parthenope, 80143 Naples, Italy (literal)
Titolo
  • Can yeast be used to study mitochondrial diseases? Biolistic tRNA mutants for the analysis of mechanisms and suppressors (literal)
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 tRNAVal) 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. (literal)
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