Document Detail

Investigation of human mitochondrial myopathies by phosphorus magnetic resonance spectroscopy.
MedLine Citation:
PMID:  4037759     Owner:  NLM     Status:  MEDLINE    
Abnormal mitochondria are an increasingly recognized cause of neuromuscular disease. We have used phosphorus magnetic resonance spectroscopy to monitor noninvasively the metabolism of high-energy phosphates and the intracellular pH of human skeletal muscle in vivo in 12 patients with mitochondrial myopathy. At rest, an abnormality could be demonstrated in 11 of 12 patients. Ten patients had evidence of a reduced muscle energy state with at least one of the following abnormalities: low phosphorylation potential, low phosphocreatine concentration, high adenosine diphosphate concentration, or high inorganic phosphate concentration. Two patients had abnormal resting muscle intracellular pH. In some patients phosphocreatine concentration decreased to low values during exercise despite limited work output. This was not accompanied by particularly severe intracellular acidosis. Evidence of impaired rephosphorylation of adenosine diphosphate to adenosine triphosphate during recovery from exercise was found in approximately half the patients. Phosphorus magnetic resonance spectroscopy is useful in the noninvasive diagnosis of mitochondrial myopathies and in defining the pathophysiological basis of these disorders.
D L Arnold; D J Taylor; G K Radda
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Annals of neurology     Volume:  18     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  1985 Aug 
Date Detail:
Created Date:  1985-10-07     Completed Date:  1985-10-07     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  189-96     Citation Subset:  IM    
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MeSH Terms
Adenosine Diphosphate / metabolism
Adenosine Triphosphate / metabolism
Hydrogen-Ion Concentration
Magnetic Resonance Spectroscopy
Metabolic Diseases / metabolism
Middle Aged
Mitochondria, Muscle* / metabolism
Muscles / metabolism*
Muscular Diseases / metabolism*
Phosphocreatine / metabolism
Phosphorus / metabolism*
Reg. No./Substance:
56-65-5/Adenosine Triphosphate; 58-64-0/Adenosine Diphosphate; 67-07-2/Phosphocreatine; 7723-14-0/Phosphorus

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

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