Abstract | BACKGROUND: METHODS: Heart homogenates from patients with valvular stenosis were tested for respiratory-chain complex II activity, lipoperoxidation, and aconitase activity by spectrophotometric assays, in the presence of reduced iron (Fe2+), oxidised iron (Fe3+), desferrioxamine, ascorbic acid, and idebenone. The Friedreich's ataxia patients (aged 11 years, 19 years, and 21 years) underwent ultrasonographic heart measurements at baseline and after 4-9 months of idebenone (5 mg/kg daily). FINDINGS: Fe2+ (but not Fe3+) decreased complex II activity and increased lipoperoxidation in heart homogenate. Addition of ascorbate or desferrioxamine increased some of the iron-induced adverse effects. Idebenone protected against these effects. In the three patients, left-ventricular mass index decreased from baseline to 4-9 months of idebenone treatment (patient 1, 145 g to 114 g; patient 2, 215 g to 151 g; patient 3, 408 g to 279 g). INTERPRETATION:
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Authors | P Rustin, J C von Kleist-Retzow, K Chantrel-Groussard, D Sidi, A Munnich, A Rötig |
Journal | Lancet (London, England)
(Lancet)
Vol. 354
Issue 9177
Pg. 477-9
(Aug 07 1999)
ISSN: 0140-6736 [Print] England |
PMID | 10465173
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Antioxidants
- Benzoquinones
- Iron Chelating Agents
- Multienzyme Complexes
- Ubiquinone
- Oxidoreductases
- Electron Transport Complex II
- Succinate Dehydrogenase
- idebenone
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Topics |
- Adult
- Antioxidants
(adverse effects, therapeutic use)
- Benzoquinones
(adverse effects, therapeutic use)
- Cardiac Volume
(drug effects)
- Cardiomyopathies
(drug therapy, pathology)
- Child
- Electron Transport Complex II
- Female
- Friedreich Ataxia
(drug therapy, pathology)
- Humans
- Iron Chelating Agents
(adverse effects, therapeutic use)
- Male
- Multienzyme Complexes
(metabolism)
- Myocardium
(pathology)
- Oxidoreductases
(metabolism)
- Succinate Dehydrogenase
(metabolism)
- Ubiquinone
(analogs & derivatives)
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