Decreased Coenzyme Q10 Levels in Multiple System Atrophy Cerebellum

http://www.ncbi.nlm.nih.gov/pubmed/27235405

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J Neuropathol Exp Neurol. 2016 Jul;75(7):663-72. doi: 10.1093/jnen/nlw037. Epub 2016 May 27.

[[ Decreased Coenzyme Q10 Levels in Multiple System Atrophy Cerebellum.]]
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Barca E1, Kleiner G1, Tang G1, Ziosi M1, Tadesse S1, Masliah E1, Louis ED1, Faust P1, Kang UJ1, Torres J1, Cortes EP1, Vonsattel JP1, Kuo SH1, Quinzii CM2.
Author information
Abstract

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 biosynthetic 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.

© 2016 American Association of Neuropathologists, Inc. All rights reserved.
KEYWORDS:

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

PMID:
    27235405
PMCID:
    PMC4913434
    [Available on 2017-07-01]
DOI:
    10.1093/jnen/nlw037

    [PubMed - in process]
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