Decreased coenzyme Q10 levels in multiple system atrophy cerebellum

Emanuele Barca, Giulio Kleiner, Guomei Tang, Marcello Ziosi, Saba Tadesse, Eliezer Masliah, Elan D. Louis, Phyllis Faust, Un J. Kang, Jose Torres, Etty P. Cortes, Jean Paul G. Vonsattel, Sheng Han Kuo, Catarina M. Quinzii

Research output: Contribution to journalArticlepeer-review

41 Scopus citations


In familial and sporadic multiple system atrophy (MSA) patients, deficiency of coenzyme Q10 (CoQ10) has been associated with mutations in COQ2, which encodes the second enzyme in the CoQ10 bio-synthetic pathway. Cerebellar ataxia is the most common presentation of CoQ10 deficiency, suggesting that the cerebellum might be selectively vulnerable to low levels of CoQ10. To investigate whether CoQ10 deficiency represents a common feature in the brains of MSA patients independent of the presence of COQ2 mutations, we studied CoQ10 levels in postmortem brains of 12 MSA, 9 Parkinson disease (PD), 9 essential tremor (ET) patients, and 12 controls. We also assessed mitochondrial respiratory chain enzyme activities, oxidative stress, mitochondrial mass, and levels of enzymes involved in CoQ biosynthesis. Our studies revealed CoQ10 deficiency in MSA cerebellum, which was associated with impaired CoQ biosynthesis and increased oxidative stress in the absence of COQ2 mutations. The levels of CoQ10 in the cerebella of ET and PD patients were comparable or higher than in controls. These findings suggest that CoQ10 deficiency may contribute to the pathogenesis of MSA. Because no disease modifying therapies are currently available, increasing CoQ10 levels by supplementation or upregulation of its biosynthesis may represent a novel treatment strategy for MSA patients.

Original languageEnglish (US)
Pages (from-to)663-672
Number of pages10
JournalJournal of neuropathology and experimental neurology
Issue number7
StatePublished - Jul 1 2016
Externally publishedYes


  • Cerebellar ataxia
  • Coenzyme Q10
  • Multiple system atrophy
  • Oxidative stress

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience


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