title: Oxidative stress induces mitochondrial fragmentation in frataxin-deficient cells.
abstract: Friedreich ataxia (FA) is the most common recessive neurodegenerative disease. It is caused by deficiency in mitochondrial frataxin, which participates in iron-sulfur cluster assembly. Yeast cells lacking frataxin (Δyfh1 mutant) showed an increased proportion of fragmented mitochondria compared to wild-type. In addition, oxidative stress induced complete fragmentation of mitochondria in Δyfh1 cells. Genetically controlled inhibition of mitochondrial fission in these cells led to increased resistance to oxidative stress. Here we present evidence that in yeast frataxin-deficiency interferes with mitochondrial dynamics, which might therefore be relevant for the pathophysiology of FA.
authors.affiliation: Mitochondria, Metals and Oxidative Stress Laboratory, Institut Jacques Monod, CNRS-Université Paris-Diderot, Sorbonne Paris Cité, 15 rue Hélène Brion, 75205 Paris cedex 13, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Lefevre
authors.forename: Dominika
authors.initials: D
authors.lastname: Sliwa
authors.forename: Pierre
authors.initials: P
authors.lastname: Rustin
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Camadro
authors.forename: Renata
authors.initials: R
authors.lastname: Santos
mesh_terms: D005621:Friedreich Ataxia / Q000235:genetics / Q000473:pathology; D020558:GTP Phosphohydrolases / Q000235:genetics; D017353:Gene Deletion; D006801:Humans; D033862:Iron-Binding Proteins / Q000235:genetics*; D008928:Mitochondria / Q000502:physiology* / Q000648:ultrastructure; D024101:Mitochondrial Proteins / Q000235:genetics; D018384:Oxidative Stress; D012441:Saccharomyces cerevisiae / Q000235:genetics / Q000502:physiology* / Q000648:ultrastructure; D029701:Saccharomyces cerevisiae Proteins / Q000235:genetics; D000096923:Frataxin
chemical_list: D020558:GTP Phosphohydrolases; D033862:Iron-Binding Proteins; D024101:Mitochondrial Proteins; D029701:Saccharomyces cerevisiae Proteins; D000096923:Frataxin; C094774:DNM1 protein, S cerevisiae