title: Mitochondrial respiratory chain function in multiple system atrophy.
abstract: Multiple system atrophy (MSA) is a clinico-pathological entity distinct from idiopathic Parkinson's disease (PD) that is responsible for 5-10% of cases of parkinsonism. Degeneration of nigral neurones is a feature of both diseases. A specific deficiency of mitochondrial complex I activity has been found in PD substantia nigra. We have analysed mitochondrial function in substantia nigra and platelets from MSA patients to identify any respiratory chain defect in this disorder and to determine its tissue specificity. As our MSA patients had been on L-DOPA, we also sought to establish whether this treatment could cause the complex I defect as seen in PD. We found no significant difference in respiratory chain activity corrected for mitochondrial mass between control and MSA patients in either of the tissues studied. These results provide a biochemical dimension to the differences between MSA and idiopathic PD. In addition, the fact that L-DOPA failed to induce a complex I defect in MSA substantia nigra suggests that this treatment is unlikely to cause the complex I deficiency in PD, without additional factors that may operate in PD.
authors.affiliation: Department of Clinical Neurosciences, Royal Free Hospital School of Medicine, London, England.
authors.forename: M
authors.initials: M
authors.lastname: Gu
authors.forename: M T
authors.initials: MT
authors.lastname: Gash
authors.forename: J M
authors.initials: JM
authors.lastname: Cooper
authors.forename: G K
authors.initials: GK
authors.lastname: Wenning
authors.forename: S E
authors.initials: SE
authors.lastname: Daniel
authors.forename: N P
authors.initials: NP
authors.lastname: Quinn
authors.forename: C D
authors.initials: CD
authors.lastname: Marsden
authors.forename: A H
authors.initials: AH
authors.lastname: Schapira
mesh_terms: D000368:Aged; D000978:Antiparkinson Agents / Q000008:administration & dosage / Q000627:therapeutic use; D001284:Atrophy / Q000473:pathology*; D001792:Blood Platelets; D001921:Brain / Q000201:enzymology / Q000473:pathology*; D002950:Citrate (si)-Synthase / Q000378:metabolism; D046508:Culture Techniques; D004579:Electron Transport; D006801:Humans; D007980:Levodopa / Q000008:administration & dosage / Q000627:therapeutic use; D008875:Middle Aged; D008928:Mitochondria / Q000201:enzymology / Q000473:pathology*; D009410:Nerve Degeneration; D010300:Parkinson Disease / Q000175:diagnosis / Q000188:drug therapy; D013378:Substantia Nigra
chemical_list: D000978:Antiparkinson Agents; D007980:Levodopa; D002950:Citrate (si)-Synthase