Serveur d'exploration sur la maladie de Parkinson

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Impaired oxidative decarboxylation of pyruvate in fibroblasts from patients with Parkinson's disease

Identifieur interne : 002435 ( Main/Exploration ); précédent : 002434; suivant : 002436

Impaired oxidative decarboxylation of pyruvate in fibroblasts from patients with Parkinson's disease

Auteurs : C. Mytilineou [États-Unis] ; P. Werner [États-Unis] ; S. Molinari [États-Unis] ; A. Di Rocco [États-Unis] ; G. Cohen [États-Unis] ; D. Yahr [États-Unis]

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RBID : ISTEX:5CCD8084F66803233DB350DE7721F9280BE048DC

Abstract

Summary: Whether or not a reported deficiency in brain mitochondrial complex I activity in Parkinson's disease represents a defect encompassing other organs or tissues has been a source of some controversy. We have examined mitochondrial respiration in fibroblasts from patients with Parkinson's disease by measuring the oxidative decarboxylation of [2-14C]pyruvate and [1,4-14C]succinate. We report that oxidation of pyruvate but not succinate was significantly reduced in fibroblasts from Parkinson patients when compared to healthy controls. These observations support the view that a widespread deficit in mitochondrial respiration exists in Parkinson's disease. Fibroblast cultures, moreover, are a source of affected proliferating cells, which can be used for in vitro studies of the nature of the respiratory defect and for testing of pharmacological interventions to correct the deficiency.

Url:
DOI: 10.1007/BF02260943


Affiliations:


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<div type="abstract" xml:lang="en">Summary: Whether or not a reported deficiency in brain mitochondrial complex I activity in Parkinson's disease represents a defect encompassing other organs or tissues has been a source of some controversy. We have examined mitochondrial respiration in fibroblasts from patients with Parkinson's disease by measuring the oxidative decarboxylation of [2-14C]pyruvate and [1,4-14C]succinate. We report that oxidation of pyruvate but not succinate was significantly reduced in fibroblasts from Parkinson patients when compared to healthy controls. These observations support the view that a widespread deficit in mitochondrial respiration exists in Parkinson's disease. Fibroblast cultures, moreover, are a source of affected proliferating cells, which can be used for in vitro studies of the nature of the respiratory defect and for testing of pharmacological interventions to correct the deficiency.</div>
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