Quinone analogs prevent enzymes targeted in Friedreich ataxia from iron-induced injury in vitro.
Unité de Recherches sur les Handicaps Génétiques de l'Enfant (INSERM U393), Hôpital des Enfants-Malades, Paris, France. rustin@necker.fr
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Rustin
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Munnich
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Rötig
D000154:Aconitate Hydratase / Q000187:drug effects* / Q000378:metabolism; D016227:Benzoquinones / Q000494:pharmacology; D003067:Coenzymes; D003676:Deferoxamine / Q000494:pharmacology; D042963:Electron Transport Complex II; D005621:Friedreich Ataxia / Q000201:enzymology*; D006801:Humans; D007501:Iron / Q000037:antagonists & inhibitors / Q000633:toxicity*; D007506:Iron-Sulfur Proteins / Q000172:deficiency / Q000187:drug effects* / Q000378:metabolism; D009097:Multienzyme Complexes / Q000187:drug effects* / Q000378:metabolism; D010088:Oxidoreductases / Q000187:drug effects* / Q000378:metabolism; D011809:Quinones / Q000494:pharmacology*; D013385:Succinate Dehydrogenase / Q000187:drug effects* / Q000378:metabolism; D014451:Ubiquinone / Q000031:analogs & derivatives / Q000494:pharmacology
D016227:Benzoquinones; D003067:Coenzymes; D007506:Iron-Sulfur Proteins; D009097:Multienzyme Complexes; D011809:Quinones; D014451:Ubiquinone; D007501:Iron; D010088:Oxidoreductases; D042963:Electron Transport Complex II; D013385:Succinate Dehydrogenase; D000154:Aconitate Hydratase; C024989:coenzyme Q10; C036619:idebenone; D003676:Deferoxamine