31P-NMR spectroscopy of skeletal muscle in Becker dystrophy and DMD/BMD carriers. Altered rate of phosphate transport

J Neurol Sci. 1992 Jun;109(2):188-95. doi: 10.1016/0022-510x(92)90167-j.

Abstract

Muscle energy metabolism was studied by phosphorus nuclear magnetic resonance spectroscopy (31P-NMR) in 6 patients with Becker dystrophy, and in 24 female DMD/BMD carriers (n = 18) and non-carriers (n = 6). At rest all patients showed a high Pi/PCr ratio due to low PCr and high Pi contents, and a high intracellular IpH. 31P-NMR of carriers and non-carriers did not differ from controls. In patients and carriers in-magnet exercise revealed a reduced ability to perform work and Pi/PCr ratios higher than controls for comparable relative levels of steady-state work. Post-exercise Pi recovery was found abnormal in patients and in carriers. The 31P-NMR abnormalities found in the working muscle of both BMD patients and female DMD/BMD carriers indicate a defect of phosphate metabolism that, be it primary or secondary, reflects a deficit of energy metabolism.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Chromosome Deletion
  • Creatine Kinase / blood
  • Dystrophin / biosynthesis
  • Dystrophin / genetics
  • Energy Metabolism
  • Exercise
  • Female
  • Heterozygote
  • Humans
  • Hydrogen-Ion Concentration
  • Magnetic Resonance Spectroscopy
  • Middle Aged
  • Muscles / chemistry*
  • Muscles / metabolism
  • Muscular Dystrophies / genetics*
  • Muscular Dystrophies / metabolism
  • Phosphates / metabolism

Substances

  • Dystrophin
  • Phosphates
  • Creatine Kinase