31P NMR spectroscopy and ergometer exercise test as evidence for muscle oxidative performance improvement with coenzyme Q in mitochondrial myopathies
| العنوان: | 31P NMR spectroscopy and ergometer exercise test as evidence for muscle oxidative performance improvement with coenzyme Q in mitochondrial myopathies |
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| المؤلفون: | G. Kozak-Ribbens, Claude Desnuelle, Sylviane Confort-Gouny, D. Vanuxem, David Bendahan, Dominique Figarella-Branger, Jean Pouget, Patrick J. Cozzone, Jean-François Pellissier, Georges Serratrice |
| المصدر: | Neurology. 42:1203-1203 |
| بيانات النشر: | Ovid Technologies (Wolters Kluwer Health), 1992. |
| سنة النشر: | 1992 |
| مصطلحات موضوعية: | Adult, Male, Mitochondrial encephalomyopathy, medicine.medical_specialty, Magnetic Resonance Spectroscopy, Adolescent, Ubiquinone, Oxidative phosphorylation, Phosphocreatine, chemistry.chemical_compound, Muscular Diseases, Mitochondrial myopathy, Internal medicine, medicine, Humans, Glycolysis, Acidosis, Chemistry, Muscles, Phosphorus, medicine.disease, Mitochondria, Muscle, Endocrinology, Biochemistry, Coenzyme Q – cytochrome c reductase, Exercise Test, Neurology (clinical), medicine.symptom, Ventilatory threshold, Oxidation-Reduction |
| الوصف: | Two patients with mitochondrial encephalomyopathy due to complexes I and IV deficiencies received 150 mg/d of coenzyme Q 10 (CoQ). We studied them with a bicycle ergometer exercise test and 31 P NMR spectroscopy before and after 10 months of treatment. Before treatment, we observed a low phosphocreatine/inorganic phosphate (PCr/P i ) resting value along with abnormally high resting lactate concentration. During exercise, there was a pronounced acidosis with delayed kinetics of postexercise recovery for blood lactate, pH, PCr, and PCr/P i ratio. Oxygen uptake during exercise was reduced while the lowering of the ventilatory threshold indicated an early activation of glycolysis. After treatment, the bicycle ergometer exercise test indicated a significant improvement with a decrease in resting blood lactate level, an increase in oxygen consumption during exercise, and an increase in the kinetics of lactate disappearance during the recovery period. A shift of the ventilatory threshold to higher workload was present. 31 P NMR spectroscopy confirmed the improvement, showing a significant increase in the PCr/P i ratio at rest and in the kinetics of recovery for pH, PCr, and PCr/P i ratio following exercise in patient 1. For patient 2, we observed a less pronounced acidosis correlated with a lesser amount of P i produced during exercise. These observations indicate an improvement of mitochondrial function and a shift from high to low glycolytic activity in both patients consequent to CoQ treatment. |
| تدمد: | 1526-632X 0028-3878 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::17ce34c8ae8f8efa0f6c97bb3ee97fa1Test https://doi.org/10.1212/wnl.42.6.1203Test |
| رقم الانضمام: | edsair.doi.dedup.....17ce34c8ae8f8efa0f6c97bb3ee97fa1 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 1526632X 00283878 |
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