| 31P NMR spectroscopy and ergometer exercise test as evidence for muscle oxidative performance improvement with coenzyme Q in mitochondrial myopathies. | |
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MedLine Citation:
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PMID: 1603348 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Two patients with mitochondrial encephalomyopathy due to complexes I and IV deficiencies received 150 mg/d of coenzyme Q10 (CoQ). We studied them with a bicycle ergometer exercise test and 31P 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. 31P 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 Pi 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. |
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Authors:
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D Bendahan; C Desnuelle; D Vanuxem; S Confort-Gouny; D Figarella-Branger; J F Pellissier; G Kozak-Ribbens; J Pouget; G Serratrice; P J Cozzone |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Neurology Volume: 42 ISSN: 0028-3878 ISO Abbreviation: Neurology Publication Date: 1992 Jun |
Date Detail:
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Created Date: 1992-07-13 Completed Date: 1992-07-13 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0401060 Medline TA: Neurology Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 1203-8 Citation Subset: AIM; IM |
Affiliation:
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Centre de Résonance Magnétique Biologique et Médicale (CRMBM), Marseille, France. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Exercise Test* Humans Magnetic Resonance Spectroscopy / diagnostic use* Male Mitochondria, Muscle* Muscles / metabolism* Muscular Diseases / diagnosis, drug therapy*, metabolism Oxidation-Reduction Phosphorus / diagnostic use Ubiquinone / therapeutic use* |
| Chemical | |
Reg. No./Substance:
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1339-63-5/Ubiquinone; 7723-14-0/Phosphorus |
| Comments/Corrections | |
Comment In:
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Neurology. 1993 Mar;43(3 Pt 1):628-9
[PMID:
8451017
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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