Journal
FRONTIERS IN NEUROSCIENCE
Volume 13, Issue -, Pages -Publisher
FRONTIERS MEDIA SA
DOI: 10.3389/fnins.2019.01187
Keywords
multiple system atrophy; parkinsonian disorders; COQ2; COQ7; ATP; coenzyme Q(10); cerebellum
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Funding
- Multiple System Atrophy Coalition
- Michael and Elizabeth Gilbert Scholarship from Neuroscience Research Australia
- Lundbeck Foundation [R248-2016-2518]
- Lundbeck Foundation [R248-2016-2518] Funding Source: researchfish
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Multiple system atrophy (MSA) is a progressive neurodegenerative disease clinically characterized by parkinsonism and cerebellar ataxia, and pathologically by oligodendrocyte alpha-synuclein inclusions. Genetic variants of COQ2 are associated with an increased risk for MSA in certain populations. Also, deficits in the level of coenzyme Q(10) and its biosynthetic enzymes are associated with MSA. Here, we measured ATP levels and expression of biosynthetic enzymes for coenzyme Q(10), including COQ2, in multiple regions of MSA and control brains. We found a reduction in ATP levels in disease-affected regions of MSA brain that associated with reduced expression of COQ2 and COQ7, supporting the concept that abnormalities in the biosynthesis of coenzyme Q(10) play an important role in the pathogenesis of MSA.
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