Document Detail

Bioenergetics of skeletal muscle in mitochondrial myopathy.
MedLine Citation:
PMID:  7707079     Owner:  NLM     Status:  MEDLINE    
31Phosphorus nuclear magnetic resonance spectroscopy was used to examine skeletal muscle in 29 patients with mitochondrial myopathy, 9 male and 20 female. Gastrocnemius was investigated in 15 patients and 30 normal subjects and finger flexor muscle (flexor digitorum superficialis, fds) in 24 patients and 35 normal controls. Both muscles were studied in 10 of the patients. Results were abnormal (outside the full range of normal values) in all but 2 patients. In 86% of patients (25/29) abnormalities were detected in resting muscle. In most cases there was a low phosphocreatine/ATP ratio, high calculated free [ADP] and low phosphorylation potential. At rest, abnormality was detected with equal ease in fds and gastrocnemius. Exercise and recovery increased the sensitivity of MRS in detecting abnormal metabolism. Finger flexion was better tolerated by patients than plantar flexion and gave bigger changes in metabolite concentrations and intracellular pH. Thus, results from fds were more easily differentiated from normal. Exercise duration was significantly shorter than in controls while phosphocreatine depletion was more rapid than normal, consistent with a shortfall in mitochondrial ATP synthesis. Nearly all patients (25/27, 93%) showed abnormalities during recovery from exercise. [ADP] was high during exercise and its recovery was delayed, providing increased drive for oxidative phosphorylation. Phosphocreatine resynthesis during recovery (which reflects oxidative ATP synthesis) was slow both in absolute terms and in relation to [ADP]. Recovery of intracellular pH after exercise was significantly more rapid than normal, consistent with an upregulation of proton efflux.(ABSTRACT TRUNCATED AT 250 WORDS)
D J Taylor; G J Kemp; G K Radda
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Publication Detail:
Type:  Clinical Trial; Journal Article    
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  127     ISSN:  0022-510X     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  1994 Dec 
Date Detail:
Created Date:  1995-05-08     Completed Date:  1995-05-08     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  198-206     Citation Subset:  IM    
MRC Biochemical and Clinical Magnetic Resonance Unit, John Radcliffe Hospital, Oxford, UK.
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MeSH Terms
Adenosine Diphosphate / metabolism
Adenosine Triphosphate / metabolism
Aged, 80 and over
DNA, Mitochondrial / metabolism
Energy Metabolism / physiology*
Exercise / physiology
Hydrogen-Ion Concentration
Magnetic Resonance Imaging
Middle Aged
Mitochondrial Myopathies / metabolism*
Muscle, Skeletal / metabolism*
Ophthalmoplegia, Chronic Progressive External / metabolism
Phosphates / metabolism
Phosphocreatine / metabolism
Reg. No./Substance:
0/DNA, Mitochondrial; 0/Phosphates; 56-65-5/Adenosine Triphosphate; 58-64-0/Adenosine Diphosphate; 67-07-2/Phosphocreatine

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