Brain, Vol 120, Issue 10 1699-1711, Copyright © 1997 by Oxford University Press
PR Barnes, GJ Kemp, DJ Taylor and GK Radda
We have used 31P magnetic resonance spectroscopy to investigate skeletal
muscle bioenergetics in a total of 31 patients with myotonic dystrophy.
Results from resting flexor digitorum superficialis and calf muscle showed
a significant elevation in the concentration ratio of inorganic phosphate
to ATP and a significant reduction in the phosphorylation potential. In
addition, in resting calf muscle the concentration ratio of phosphocreatine
to ATP was reduced, and the resting intracellular pH and calculated free
cytosolic ADP concentration were elevated. In general, the abnormalities
observed were more marked in those patients who were more severely affected
as judged by their ability to exercise. During aerobic exercise in both
calf muscle and flexor digitorum superficialis, phosphocreatine was
depleted more rapidly in patients than in control subjects but the muscle
acidified less and ADP concentrations were higher. Calculated ATP turnover
was significantly elevated. Analysis of the recovery kinetics for
phosphocreatine following exercise provides evidence for a small but
significant reduction in mitochondrial function. Analysis of the response
of flexor digitorum superficialis to ischaemic exercise provides evidence
of a reduction in the relative utilization of glycogen to produce ATP which
may account, in part, for the reduced acidification seen in exercising
muscle in myotonic dystrophy. There was no definite evidence of an
alteration in proton handling capacity in this condition.
ARTICLES
Skeletal muscle metabolism in myotonic dystrophy A 31P magnetic resonance spectroscopy study
MRC Biochemical and Clinical Magnetic Resonance Unit, Oxford Radcliffe Hospital, UK.
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