Dj. Taylor et al., A P-31 MAGNETIC-RESONANCE SPECTROSCOPY STUDY OF MITOCHONDRIAL-FUNCTION IN SKELETAL-MUSCLE OF PATIENTS WITH PARKINSONS-DISEASE, Journal of the neurological sciences, 125(1), 1994, pp. 77-81
The activity of complex I of the respiratory chain is decreased in the
substantia nigra of patients with Parkinson's disease (PD) but the pr
esence of this defect in skeletal muscle is controversial. Therefore,
the mitochondrial function of skeletal muscle in patients with PD was
investigated in vivo using P-31 magnetic resonance spectroscopy. Resul
ts from 7 PD patients, 11 age matched controls and 9 mitochondrial myo
pathy patients with proven complex I deficiency were obtained from fin
ger flexor muscle at rest, during exercise and in recovery from exerci
se. In resting muscle, the patients with mitochondrial myopathy showed
a low PCr/ATP ratio, a low phosphorylation potential, a high P-i/PCr
ratio and a high calculated free [ADP]. During exercise, stores of hig
h energy phosphate were depleted more rapidly than normal, while in re
covery, the concentration of phosphocreatine and free ADP returned to
pre-exercise values more slowly than normal. In contrast, the patients
with PD were not significantly different from normal for any of these
variables, and no abnormality of muscle energetics was detected. Thre
e of the PD patients also had mitochondrial function assessed biochemi
cally in muscle biopsies. No respiratory chain defect was identified i
n any of these patients by polarography or enzyme analysis when compar
ed with age-matched controls. These results suggest that skeletal musc
le is not a suitable tissue for the investigation and identification o
f the biochemical basis of the nigral complex I deficiency in PD.