A 31P magnetic resonance spectroscopy study of mitochondrial function in skeletal muscle of patients with Parkinson's disease.
The activity of complex I of the respiratory chain is decreased in the substantia nigra of patients with Parkinson's disease (PD) but the presence of this defect in skeletal muscle is controversial. Therefore, the mitochondrial function of skeletal muscle in patients with PD was investigated in vivo using 31P magnetic resonance spectroscopy. Results from 7 PD patients, 11 age matched controls and 9 mitochondrial myopathy patients with proven complex I deficiency were obtained from finger flexor muscle at rest, during exercise and in recovery from exercise. 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 high energy phosphate were depleted more rapidly than normal, while in recovery, 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. Three of the PD patients also had mitochondrial function assessed biochemically in muscle biopsies. No respiratory chain defect was identified in any of these patients by polarography or enzyme analysis when compared with age-matched controls. These results suggest that skeletal muscle is not a suitable tissue for the investigation and identification of the biochemical basis of the nigral complex I deficiency in PD.
Taylor
D J
DJ
MRC-Biochemical and Clinical Magnetic Resonance Unit, John Radcliffe Hospital, Oxford, UK.
Krige
D
D
Barnes
P R
PR
Kemp
G J
GJ
Carroll
M T
MT
Mann
V M
VM
Cooper
J M
JM
Marsden
C D
CD
Schapira
A H
AH
D000293:Adolescent; D000328:Adult; D000368:Aged; D001792:Blood Platelets / Q000502:physiology; D002648:Child; D004734:Energy Metabolism; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008875:Middle Aged; D008931:Mitochondria, Muscle / Q000502:physiology*; D018482:Muscle, Skeletal / Q000503:physiopathology*; D016660:NAD(P)H Dehydrogenase (Quinone) / Q000172:deficiency; D010300:Parkinson Disease / Q000503:physiopathology*; D010758:Phosphorus; D005082:Physical Exertion; D012016:Reference Values; D012146:Rest
D010758:Phosphorus; D016660:NAD(P)H Dehydrogenase (Quinone)