title: Impaired oxidative decarboxylation of pyruvate in fibroblasts from patients with Parkinson's disease.
abstract: 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.
authors.affiliation: Department of Neurology, Mount Sinai School of Medicine, New York, NY, USA.
authors.forename: C
authors.initials: C
authors.lastname: Mytilineou
authors.forename: P
authors.initials: P
authors.lastname: Werner
authors.forename: S
authors.initials: S
authors.lastname: Molinari
authors.forename: A
authors.initials: A
authors.lastname: Di Rocco
authors.forename: G
authors.initials: G
authors.lastname: Cohen
authors.forename: M D
authors.initials: MD
authors.lastname: Yahr
mesh_terms: D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D002478:Cells, Cultured; D003653:Decarboxylation; D005069:Evaluation Studies as Topic; D005260:Female; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000378:metabolism*; D010084:Oxidation-Reduction; D010101:Oxygen Consumption / Q000502:physiology*; D010300:Parkinson Disease / Q000175:diagnosis / Q000378:metabolism*; D011773:Pyruvates / Q000378:metabolism*; D019289:Pyruvic Acid; D013386:Succinates / Q000378:metabolism; D019802:Succinic Acid
chemical_list: D011773:Pyruvates; D013386:Succinates; D019289:Pyruvic Acid; D019802:Succinic Acid