A primary mitochondrial dysfunction has been recently hypothesized in the pathogenesis of Parkinson's disease (PD). As a matter of fact, MPTP (a drug able to induce neuronal loss in substantia nigra) is a specific inhibitor of Complex I, while controversial data on Complex I deficiency have been reported in platelets, muscle and brain of patients affected by Parkinson's disease. Here we report data on respiratory chain enzyme activities in isolated muscle mitochondria from 6 patients with classical PD (two with the hereditary form, HPD), and in 6 patients with a multisystem extrapyramidal disorder (MSED) with parkinsonsim as the main clinical feature. While histological and biochemical evidence of mitochondrial dysfunction has been detected in 1 case with PD and in 3 cases with MSED, normal results have been obtained in the other cases confirming the extreme clinical and biochemical heterogeneity of these disorders.

Manneschi, L., Dotti, M., Battisti, C., DE STEFANO, N., Federico, A. (1994). Muscle respiratory chain enzyme activities in parkinson's disease and in multisystem extrapyramidal disorders with parkinsonism as the main clinical feature. ARCHIVES OF GERONTOLOGY AND GERIATRICS, 19(Suppl 1), 155-161 [10.1016/S0167-4943(05)80060-3].

Muscle respiratory chain enzyme activities in parkinson's disease and in multisystem extrapyramidal disorders with parkinsonism as the main clinical feature

DOTTI, M.;BATTISTI, C.;DE STEFANO, N.;FEDERICO, A.
1994-01-01

Abstract

A primary mitochondrial dysfunction has been recently hypothesized in the pathogenesis of Parkinson's disease (PD). As a matter of fact, MPTP (a drug able to induce neuronal loss in substantia nigra) is a specific inhibitor of Complex I, while controversial data on Complex I deficiency have been reported in platelets, muscle and brain of patients affected by Parkinson's disease. Here we report data on respiratory chain enzyme activities in isolated muscle mitochondria from 6 patients with classical PD (two with the hereditary form, HPD), and in 6 patients with a multisystem extrapyramidal disorder (MSED) with parkinsonsim as the main clinical feature. While histological and biochemical evidence of mitochondrial dysfunction has been detected in 1 case with PD and in 3 cases with MSED, normal results have been obtained in the other cases confirming the extreme clinical and biochemical heterogeneity of these disorders.
1994
Manneschi, L., Dotti, M., Battisti, C., DE STEFANO, N., Federico, A. (1994). Muscle respiratory chain enzyme activities in parkinson's disease and in multisystem extrapyramidal disorders with parkinsonism as the main clinical feature. ARCHIVES OF GERONTOLOGY AND GERIATRICS, 19(Suppl 1), 155-161 [10.1016/S0167-4943(05)80060-3].
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11365/24301
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