Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria

Deletion of YDL120, the yeast homologue of the human gene responsible for Friedreich's ataxia, elicits decreased cellular respiration associated with decreased cytochrome c oxidase activity and, in certain nuclear backgrounds, mitochondrial DNA is lost. In the null mutants, the cellular growth...

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Bibliographic Details
Published inFEBS letters Vol. 411; no. 2; pp. 373 - 377
Main Authors Foury, Françoise, Cazzalini, Ornella
Format Journal Article
LanguageEnglish
Published England Elsevier B.V 14.07.1997
Subjects
ABC, ATP-binding cassette
Adenosine Triphosphatases - genetics
Adenosine Triphosphatases - metabolism
Animals
Cell Division - drug effects
Cell Respiration
Copper
Copper - pharmacology
DNA, Mitochondrial - analysis
Frataxin
Friedreich Ataxia - etiology
Friedreich Ataxia - genetics
Friedreich's ataxia
Gene Deletion
Gene disruption
Genes, Fungal
Gentamicins - pharmacology
Humans
Hydrogen Peroxide - pharmacology
Iron
Iron - metabolism
Iron - pharmacology
Iron-Binding Proteins
Meiosis
Mitochondria
Mitochondria - metabolism
Molecular Sequence Data
mtDNA, mitochondrial DNA
Mutagenesis
PCR, polymerase chain reaction
Phosphotransferases (Alcohol Group Acceptor) - genetics
Polymerase Chain Reaction
Rats
Saccharomyces cerevisiae - genetics
Yeast homologue
Yeast homologue
Friedreich's ataxia
Mitochondria
Gene disruption
PCR, polymerase chain reaction
Iron
mtDNA, mitochondrial DNA
Copper
ABC, ATP-binding cassette
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Summary:Deletion of YDL120, the yeast homologue of the human gene responsible for Friedreich's ataxia, elicits decreased cellular respiration associated with decreased cytochrome c oxidase activity and, in certain nuclear backgrounds, mitochondrial DNA is lost. In the null mutants, the cellular growth is highly sensitive to oxidants, such as H 2O 2, iron and copper. However, only ferrous sulfate elicits loss of mitochondrial DNA. Mitochondria of the null mutants contain 10 times more iron than wild-type. The neurodegeneration observed in Friedreich's ataxia can be well explained on the basis of a mitochondrial iron overload responsible for an increased production of highly toxic free radicals.
Bibliography:ObjectType-Article-2
SourceType-Scholarly Journals-1
ObjectType-Feature-1
content type line 23
ISSN:0014-5793
1873-3468
DOI:10.1016/S0014-5793(97)00734-5