Document Detail


31P NMR spectroscopy and ergometer exercise test as evidence for muscle oxidative performance improvement with coenzyme Q in mitochondrial myopathies.
MedLine Citation:
PMID:  1603348     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
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:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Neurology     Volume:  42     ISSN:  0028-3878     ISO Abbreviation:  Neurology     Publication Date:  1992 Jun 
Date Detail:
Created Date:  1992-07-13     Completed Date:  1992-07-13     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1203-8     Citation Subset:  AIM; IM    
Affiliation:
Centre de Résonance Magnétique Biologique et Médicale (CRMBM), Marseille, France.
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MeSH Terms
Descriptor/Qualifier:
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:
1339-63-5/Ubiquinone; 7723-14-0/Phosphorus
Comments/Corrections
Comment In:
Neurology. 1993 Mar;43(3 Pt 1):628-9   [PMID:  8451017 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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