Mitochondrial complex I deficiency in Parkinson's disease.
The structure and function of mitochondrial respiratory-chain enzyme proteins were studied postmortem in the substantia nigra of nine patients with Parkinson's disease and nine matched controls. Total protein and mitochondrial mass were similar in the two groups. NADH-ubiquinone reductase (Complex I) and NADH cytochrome c reductase activities were significantly reduced, whereas succinate cytochrome c reductase activity was normal. These results indicated a specific defect of Complex I activity in the substantia nigra of patients with Parkinson's disease. This biochemical defect is the same as that produced in animal models of parkinsonism by 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) and adds further support to the proposition that Parkinson's disease may be due to an environmental toxin with action(s) similar to those of MPTP.
Schapira
A H
AH
Department of Neurological Science, Royal Free Hospital School of Medicine, London, England.
Cooper
J M
JM
Dexter
D
D
Clark
J B
JB
Jenner
P
P
Marsden
C D
CD
D042963:Electron Transport Complex II; D014450:Electron Transport Complex III / Q000378:metabolism; D006801:Humans; D008928:Mitochondria / Q000378:metabolism*; D009097:Multienzyme Complexes / Q000378:metabolism; D016660:NAD(P)H Dehydrogenase (Quinone); D010088:Oxidoreductases / Q000378:metabolism; D010300:Parkinson Disease / Q000378:metabolism*; D011808:Quinone Reductases / Q000172:deficiency*; D013378:Substantia Nigra / Q000378:metabolism; D013385:Succinate Dehydrogenase / Q000378:metabolism
D009097:Multienzyme Complexes; D010088:Oxidoreductases; D042963:Electron Transport Complex II; D013385:Succinate Dehydrogenase; D016660:NAD(P)H Dehydrogenase (Quinone); D011808:Quinone Reductases; D014450:Electron Transport Complex III