Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia.

Rötig, A; de Lonlay, P; Chretien, D; Foury, F; Koenig, M; Sidi, D; Munnich, A; Rustin, P

Rötig, A; de Lonlay, P; Chretien, D; Foury, F; Koenig, M; Sidi, D; Munnich, A; Rustin, P.

Affiliation

Rötig A; INSERM U393, Département de Génétique, Hôpital Necker-Enfants-Malades, Paris, France.

Nat Genet ; 17(2): 215-7, 1997 Oct.

Article in En | MEDLINE | ID: mdl-9326946

Subject(s)

Aconitate Hydratase/deficiency; Friedreich Ataxia/metabolism; Iron-Binding Proteins; Iron-Sulfur Proteins/deficiency; Mitochondria/metabolism; Citric Acid Cycle; Electron Transport; Endocardium/metabolism; Friedreich Ataxia/genetics; Genes, Fungal; Humans; Iron/metabolism; Models, Biological; Mutation; Myocardium/metabolism; Oxidative Stress; Phosphotransferases (Alcohol Group Acceptor)/genetics; Phosphotransferases (Alcohol Group Acceptor)/metabolism; Saccharomyces cerevisiae/genetics; Saccharomyces cerevisiae/metabolism; Frataxin

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Collection: 01-internacional Database: MEDLINE Main subject: Aconitate Hydratase / Friedreich Ataxia / Iron-Binding Proteins / Iron-Sulfur Proteins / Mitochondria Limits: Humans Language: En Journal: Nat Genet Journal subject: GENETICA MEDICA Year: 1997 Type: Article Affiliation country: France

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