Muscle energy metabolism in female DMD/BMD carriers: a 31P-MR spectroscopy study.
31P nuclear magnetic resonance spectroscopy (31P-MRS) was used to investigate the energy metabolism of the gastrocnemius muscle in 16 DMD/BMD female carriers. No significant difference was found with the controls in the resting muscle, while all carriers showed a decreased ability to perform work, and a higher Pi/PCr ratio for comparable relative levels of steady-state work. The rate of postexercise recovery of PCr/Pi ratio was lower in all carriers. The decreased rate of PCr/Pi recovery appears to be mainly due to a decreased rate of Pi recovery. Our findings show abnormal muscle energy metabolism in DMD/BMD female carriers.
Barbiroli
B
B
Istituto di Clinica Neurologica, Università di Bologna, Italy.
Funicello
R
R
Ferlini
A
A
Montagna
P
P
Zaniol
P
P
D000255:Adenosine Triphosphate / Q000378:metabolism; D000293:Adolescent; D000328:Adult; D004734:Energy Metabolism / Q000502:physiology; D005080:Exercise Test; D005260:Female; D006579:Heterozygote; D006801:Humans; D006863:Hydrogen-Ion Concentration; D009682:Magnetic Resonance Spectroscopy; D008875:Middle Aged; D009119:Muscle Contraction / Q000502:physiology; D009132:Muscles / Q000378:metabolism*; D009136:Muscular Dystrophies / Q000235:genetics* / Q000378:metabolism; D010710:Phosphates / Q000378:metabolism; D010725:Phosphocreatine / Q000378:metabolism
D010710:Phosphates; D010725:Phosphocreatine; D000255:Adenosine Triphosphate