Respiratory chain and mitochondrial DNA in muscle and brain in Parkinson's disease patients.

TitleRespiratory chain and mitochondrial DNA in muscle and brain in Parkinson's disease patients.
Publication TypeJournal Article
Year of Publication1993
AuthorsDiDonato, S, Zeviani, M, Giovannini, P, Savarese, N, Rimoldi, M, Mariotti, C, Girotti, F, Caraceni, T
JournalNeurology
Volume43
Issue11
Pagination2262-8
Date Published1993 Nov
ISSN0028-3878
KeywordsAdult, Aged, Brain Chemistry, DNA, Mitochondrial, Electron Transport, Female, Humans, Male, Middle Aged, Mitochondria, Muscle, Parkinson Disease, Polymerase Chain Reaction
Abstract

There are several reports of a defect of complex I in the substantia nigra (SN) of Parkinson's disease (PD) patients. To evaluate whether this is specific to dopaminergic neurons or the phenotypically relevant consequence of a widespread failure of the mitochondrial oxidative phosphorylation (OXPHOS) system, we measured respiratory enzyme activities in muscle homogenates from 16 PD patients and eight age-matched controls, and in muscle isolated mitochondria of six PD patients and six age-matched controls. We found no difference between the PD and control groups. In addition, we detected, by polymerase chain reaction, the mitochondrial DNA (mtDNA) "common deletion" (CD) in muscle specimens of 14 of 17 PD patients, but we obtained similar results in age-matched controls. In both groups, the amount of CD-specific deleted (delta) mtDNA ranged from 0.0% to 0.1%. Our data suggest that PD cannot be attributed to a multisystem decline of mitochondrial OXPHOS, and that lesions of muscle mtDNA in PD are likely due to normal aging. However, there was a remarkable accumulation of delta mtDNA in the SN of a PD patient and an age-matched control, suggesting that the SN is exquisitely sensitive to age-dependent damage of the mitochondrial genome.

DOI10.1212/wnl.43.11.2262
Alternate JournalNeurology
Citation Key10.1212/wnl.43.11.2262
PubMed ID8232940