Document Detail


Bioenergetics of skeletal muscle in mitochondrial myopathy.
MedLine Citation:
PMID:  7707079     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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)
Authors:
D J Taylor; G J Kemp; G K Radda
Related Documents :
3407759 - G-1,6-p2, glycolysis, and energy metabolism during circulatory occlusion in human skele...
8175559 - 31p-nmr spectroscopy, rsemg, and histochemical fiber types of human wrist flexor muscles.
16794019 - Changes in tissue water content measured with multiple-frequency bioimpedance and metab...
8760079 - Metabolic response of type i and ii muscle fibers during repeated bouts of maximal exer...
11355779 - Comparison of 99mtc-tetrofosmin uptakes on planar images with those in excised rats org...
2537939 - Effects of anoxia and recovery on the neurotransmission and level of high-energy phosph...
12527969 - The effect of intermittent training in hypobaric hypoxia on sea-level exercise: a cross...
870979 - A multifaceted approach to prevention of thromboembolism: a report of 529 cases.
24022569 - Effect of aerobic exercise on mirna-tlr4 signaling in atherosclerosis.
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    
Affiliation:
MRC Biochemical and Clinical Magnetic Resonance Unit, John Radcliffe Hospital, Oxford, UK.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Adenosine Diphosphate / metabolism
Adenosine Triphosphate / metabolism
Adolescent
Adult
Aged
Aged, 80 and over
Child
DNA, Mitochondrial / metabolism
Energy Metabolism / physiology*
Exercise / physiology
Female
Humans
Hydrogen-Ion Concentration
Magnetic Resonance Imaging
Male
Middle Aged
Mitochondrial Myopathies / metabolism*
Muscle, Skeletal / metabolism*
Ophthalmoplegia, Chronic Progressive External / metabolism
Phosphates / metabolism
Phosphocreatine / metabolism
Chemical
Reg. No./Substance:
0/DNA, Mitochondrial; 0/Phosphates; 56-65-5/Adenosine Triphosphate; 58-64-0/Adenosine Diphosphate; 67-07-2/Phosphocreatine

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


Previous Document:  Corticonigral degeneration with neuronal achromasia presenting with primary progressive aphasia: ult...
Next Document:  Length of hospital stay for cerebrovascular disease in the United States: Professional Activity Stud...