Heart hypertrophy and function are improved by idebenone in Friedreich's ataxia

Free Radical Research

Volumn 36, Issue 4, 2002, Pages 467-469

  Rustin, Pierre ^a   Rötig, Agnès ^a   Munnich, Arnold ^a   Sidi, Daniel ^a  

^a HÔPITAL NECKER ENFANTS MALADES   (France)

Author keywords

Ataxia; Frataxin; Hypertrophic cardiomyopathy; Idebenone

Indexed keywords

FRATAXIN; IDEBENONE; IRON; PLACEBO; QUINONE DERIVATIVE; SCAVENGER; SULFUR; SUPEROXIDE;

ADOLESCENT; ADULT; ARTICLE; CELL COMPOSITION; CELL ORGANELLE; CELL PROTECTION; CHILD; CLINICAL ARTICLE; CLINICAL TRIAL; CONTROLLED CLINICAL TRIAL; CONTROLLED STUDY; DEGENERATIVE DISEASE; DISEASE COURSE; DISEASE SEVERITY; DOSE RESPONSE; DRUG EFFECT; ECHOCARDIOGRAPHY; FEMALE; FRIEDREICH ATAXIA; HEART FUNCTION; HEART LEFT VENTRICLE MASS; HEART VENTRICLE FUNCTION; HUMAN; HYPERTROPHIC CARDIOMYOPATHY; INTRON; MALE; MITOCHONDRION; OXIDATIVE STRESS; PATIENT CARE; PRESCRIPTION; PROTEIN FUNCTION; SIDE EFFECT; TREATMENT OUTCOME; TRINUCLEOTIDE REPEAT;

ADMINISTRATION, ORAL; ADOLESCENT; ADULT; ANTIOXIDANTS; BENZOQUINONES; CARDIOMYOPATHY, HYPERTROPHIC; CARRIER PROTEINS; CHILD; CHILD, PRESCHOOL; FEMALE; FRIEDREICH ATAXIA; HUMANS; IRON-BINDING PROTEINS; MALE; TREATMENT OUTCOME; VENTRICULAR FUNCTION, LEFT;

ATAXIA;

EID: 0036554660     PISSN: 10715762     EISSN: None     Source Type: Journal    
DOI: 10.1080/10715760290021333     Document Type: Article

References (7)

1. Campuzano, V., Montermini, L., Molto, M.D., et al., (1996) "Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion", Science 271, 1423-1427.
2. Babcock, M., de Silva, D., Oaks, R., et al., (1997) "Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin", Science 276, 1709-1712.
3. Foury, F. and Cazzalini, O. (1997) "Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria", FEBS Lett. 411, 373-377.
4. Rötig, A., de Lonlay, P., Chretien, D., et al., (1997) "Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia", Nat. Genet. 17, 215-217.
5. Puccio, H., Simon, D., Cossée, M., et al., (2001) "Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits", Nat. Genet. 27, 181-186.
6. Rustin, P., von Kleist-Retzow, J.C., Chantrel-Groussard, K., et al., (1999) "Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study", Lancet 354, 477-479.
7. Siwik, D.A., Tzortzis, J.D., Pimental, D.R., et al., (1999) "Inhibition of copper-zinc superoxide dismutase induces cell growth, hypertrophic phenotype, and apoptosis in neonatal rat cardiac myocytes in vitro", Circ. Res. 85, 147-153.