title: Anatomic and disease specificity of NADH CoQ1 reductase (complex I) deficiency in Parkinson's disease.
abstract: 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) is thought to produce parkinsonism in humans and other primates through its inhibition of complex I. The recent discovery of mitochondrial complex I deficiency in the substantia nigra of patients with Parkinson's disease has provided a remarkable link between the idiopathic disease and the action of the neurotoxin MPTP. This article shows that complex I deficiency in Parkinson's disease is anatomically specific for the substantia nigra, and is not present in another neurodegenerative disorder involving the substantia nigra. Evidence is also provided to show that there is no correlation between L-3,4-dihydroxyphenylalanine therapy and complex I deficiency. These results suggest that complex I deficiency may be the underlying cause of dopaminergic cell death in Parkinson's disease.
authors.affiliation: Department of Neurological Science, Royal Free Hospital School of Medicine, London, England.
authors.forename: A H
authors.initials: AH
authors.lastname: Schapira
authors.forename: V M
authors.initials: VM
authors.lastname: Mann
authors.forename: J M
authors.initials: JM
authors.lastname: Cooper
authors.forename: D
authors.initials: D
authors.lastname: Dexter
authors.forename: S E
authors.initials: SE
authors.lastname: Daniel
authors.forename: P
authors.initials: P
authors.lastname: Jenner
authors.forename: J B
authors.initials: JB
authors.lastname: Clark
authors.forename: C D
authors.initials: CD
authors.lastname: Marsden
mesh_terms: D002950:Citrate (si)-Synthase / Q000378:metabolism; D006801:Humans; D016660:NAD(P)H Dehydrogenase (Quinone); D010300:Parkinson Disease / Q000201:enzymology*; D011808:Quinone Reductases / Q000172:deficiency*; D013378:Substantia Nigra / Q000201:enzymology
chemical_list: D016660:NAD(P)H Dehydrogenase (Quinone); D011808:Quinone Reductases; D002950:Citrate (si)-Synthase