Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia

Heart

Volumn 87, Issue 4, 2002, Pages 346-349

  Hausse, A O ^a   Aggoun, Y ^a   Bonnet, D ^a   Sidi, D ^a   Munnich, Arnold ^a   Rotig A ^a   Rustin, P ^a  

^a HÔPITAL NECKER ENFANTS MALADES   (France)

Author keywords

[No Author keywords available]

Indexed keywords

FRATAXIN; IDEBENONE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); SUCCINATE DEHYDROGENASE (UBIQUINONE); TRINUCLEOTIDE; UBIQUINOL CYTOCHROME C REDUCTASE;

ADOLESCENT; ADULT; ANTIOXIDANT ACTIVITY; ARTICLE; CHILD; CLINICAL ARTICLE; CLINICAL TRIAL; ENZYME DEFICIENCY; FEMALE; FRIEDREICH ATAXIA; HEART LEFT VENTRICLE MASS; HEART PROTECTION; HEART VENTRICLE HYPERTROPHY; HUMAN; HYPERTROPHIC CARDIOMYOPATHY; MALE; MITOCHONDRIAL RESPIRATION; OXIDATIVE STRESS; PRIORITY JOURNAL; PROTEIN FUNCTION; RESPIRATORY CHAIN; SIDE EFFECT; WEIGHT GAIN;

EID: 0036221156     PISSN: 13556037     EISSN: None     Source Type: Journal    
DOI: 10.1136/heart.87.4.346     Document Type: Article

References (29)

1. Friedreich's ataxia: A clinical and genetic study of 90 families with an analysis of early diagnostic criteria and interfamilial clustering of clinical features
2. Clinical and genetic abnormalities in patients with Friedreich's ataxia
3. The varying evolution of Friedreich's ataxia cardiomyopathy
4. Mapping of mutation causing Friedreich's ataxia to human chromosome 9
5. Friedreich's ataxia: Autosomal recessive disease caused by an intronic GAA triplet repeat expansion
6. Molecular pathogenesis of Friedreich ataxia
7. Friedreich ataxia: An overview
8. Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia
9. Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin
10. Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria
11. Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue
12. Clinical presentation of respiratory chain deficiency
13. Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression
14. Inhibition of copper-zinc superoxide dismutase induces cell growth, hypertrophic phenotype, and apoptosis in neonatal rat cardiac myocytes in vitro
15. Quinone analogs prevent enzymes targeted in Friedreich ataxia from iron-induced injury in vitro
16. Effect of idebenone on cardiomyopathy in Friedreich's ataxia: A preliminary study
17. ACC/AHA Guidelines for the Clinical Application of Echocardiography. A report of the American College of Cardiology/American Heart Association task force on practice guidelines (committee on clinical application of echocardiography). Developed in collaboration with the American Society of Echocardiography
18. Echocardiographic determination of left ventricular mass in man. Anatomic validation of the method
19. Chemistry, toxicology, pharmacology and pharmacokinetics of idebenone; a review
20. Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation
21. Deferoxamine fails to improve postischemic cardiac function in hypertrophied hearts
22. Normal serum iron and ferritin concentrations in patients with Friedreich's ataxia
23. Vitamin C and iron
24. Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency
25. Oxidative stress in patients with Friedreich ataxia
26. Marked variation in the cardiomyopathy associated with Friedreich's ataxia
27. Biventricular function in Friedreich's ataxia: A radionuclide angiographic study
28. Cardiac angiographic findings in Friedreich's ataxia
29. Antioxidant treatment improves in vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich's ataxia