title: Coenzyme Q10 Levels Are Decreased in the Cerebellum of Multiple-System Atrophy Patients.
abstract: BACKGROUND
  The objective of this study was to evaluate whether the levels of coenzyme Q10 (CoQ10) in brain tissue of multiple system atrophy (MSA) patients differ from those in elderly controls and in patients with other neurodegenerative diseases.
  
  
  METHODS
  Flash frozen brain tissue of a series of 20 pathologically confirmed MSA patients [9 olivopontocerebellar atrophy (OPCA) type, 6 striatonigral degeneration (SND) type, and 5 mixed type] was used for this study. Elderly controls (n = 37) as well as idiopathic Parkinson's disease (n = 7), dementia with Lewy bodies (n = 20), corticobasal degeneration (n = 15) and cerebellar ataxia (n = 18) patients were used as comparison groups. CoQ10 was measured in cerebellar and frontal cortex tissue by high performance liquid chromatography.
  
  
  RESULTS
  We detected a statistically significant decrease (by 3-5%) in the level of CoQ10 in the cerebellum of MSA cases (P = 0.001), specifically in OPCA (P = 0.001) and mixed cases (P = 0.005), when compared to controls as well as to other neurodegenerative diseases [dementia with Lewy bodies (P<0.001), idiopathic Parkinson's disease (P<0.001), corticobasal degeneration (P<0.001), and cerebellar ataxia (P = 0.001)].
  
  
  CONCLUSIONS
  Our results suggest that a perturbation in the CoQ10 biosynthetic pathway is associated with the pathogenesis of MSA but the mechanism behind this finding remains to be elucidated.
authors.affiliation: Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, United Kingdom.
authors.forename: Lucia V
authors.initials: LV
authors.lastname: Schottlaender
authors.affiliation: Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, United Kingdom.|Department of Clinical and Experimental Epilepsy, UCL Institute of Neurology, Queen Square, London, United Kingdom.
authors.forename: Conceição
authors.initials: C
authors.lastname: Bettencourt
authors.affiliation: Reta Lila Weston Institute for Neurological Studies and The Queen Square Brain Bank, London, United Kingdom.
authors.forename: Aoife P
authors.initials: AP
authors.lastname: Kiely
authors.affiliation: Neurometabolic Unit, National Hospital, London, United Kingdom.
authors.forename: Annapurna
authors.initials: A
authors.lastname: Chalasani
authors.affiliation: Neurometabolic Unit, National Hospital, London, United Kingdom.
authors.forename: Viruna
authors.initials: V
authors.lastname: Neergheen
authors.affiliation: Reta Lila Weston Institute for Neurological Studies and The Queen Square Brain Bank, London, United Kingdom.
authors.forename: Janice L
authors.initials: JL
authors.lastname: Holton
authors.affiliation: Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, United Kingdom.|Neurometabolic Unit, National Hospital, London, United Kingdom.
authors.forename: Iain
authors.initials: I
authors.lastname: Hargreaves
authors.affiliation: Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, United Kingdom.|Neurogenetics Laboratory, The National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.|The MRC Centre for Neuromuscular Diseases, UCL Institute of Neurology, London, United Kingdom.
authors.forename: Henry
authors.initials: H
authors.lastname: Houlden
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000375:Aging / Q000378:metabolism; D002531:Cerebellum / Q000378:metabolism*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000378:metabolism*; D019636:Neurodegenerative Diseases / Q000378:metabolism; D014451:Ubiquinone / Q000378:metabolism*
chemical_list: D014451:Ubiquinone; C025204:Ubiquinone Q2