A quantitative study of bioenergetics in skeletal muscle lacking utrophin and dystrophin

  • Cole M
  • Rafael J
  • Taylor D
 et al. 
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Muscle energetics and function were investigated in the hindlimb of mice lacking dystrophin (mdx), utrophin and dystrophin (utr-dys) and controls (C57Bl/10) using31P and1H magnetic resonance techniques, electrical nerve stimulation and direct biochemical analysis. At rest, [adenosine triphosphate] and [total creatine] were lowest in utr-dys, while [inorganic phosphate] was elevated. Calculated [adenosine diphosphate] was 3-fold higher in mdx and 5-fold higher in utr-dys than in controls, consistent with an increased adenosine triphosphate requirement for ion pump activity. During stimulation, force production was low only in utr-dys, and this was reflected in the bioenergetic changes. Initial recovery rates of [phosphocreatine] and [adenosine diphosphate] after stimulation were rapid in all groups, indicative of normal mitochondrial adenosine triphosphate production in utr-dys and mdx. Recovery of pH was slow in utr-dys. The data indicate that the severe abnormalities which are present in the absence of utrophin and dystrophin leave basic muscle energetics intact and appear confined to processes involving the sarcolemma. © 2002 Elsevier Science B.V. All rights reserved.

Author-supplied keywords

  • Dystrophin
  • Energy metabolism
  • Magnetic resonance imaging
  • Magnetic resonance spectroscopy
  • Skeletal muscle
  • Utrophin

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  • M. A. Cole

  • J. A. Rafael

  • D. J. Taylor

  • R. Lodi

  • K. E. Davies

  • P. Styles

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