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Abstract
BackgroundThe 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.MethodsFlash 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.ResultsWe 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)].ConclusionOur 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.
Highlights
Multiple system atrophy (MSA) is a fatal late onset neurodegenerative condition
Our results suggest that a perturbation in the coenzyme Q10 (CoQ10) biosynthetic pathway is associated with the pathogenesis of multiple system atrophy (MSA) but the mechanism behind this finding remains to be elucidated
Glossary: MSA = multiple system atrophy; MSA_SND = MSA striatonigral degeneration; MSA_mixed = MSA mixed; MSA_OPCA = MSA olivopontocerebellar atrophy; CBD = corticobasal degeneration; DLB = dementia with Lewy bodies; IPD = idiopathic Parkinson’s disease; CRB_ATX = cerebellar ataxia; SCA = spinocerebellar ataxia; FRDA = Friedreich’s ataxia; other_ataxias = other ataxias of miscellaneous origin; OR = odds ratio; 95% CI = 95% confidence interval. doi:10.1371/journal.pone.0149557.t002
Summary
MSA is characterized by parkinsonism, cerebellar ataxia and autonomic dysfunction. It presents with widespread alpha-synuclein immunoreactive glial cytoplasmic inclusions and neurodegeneration [1], and can be subdivided into 3 neuropathological subtypes according to the affected brain regions. These subtypes are: 1) olivopontocerebellar atrophy (OPCA), 2) striatonigral degeneration (SND), and 3) a combination of the two, referred to as mixed [2]. 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
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