Objective: To assess CSF levels of coenzyme Q10 in multiple system atrophy (MSA) vs. Parkinson’s disease (PD), progressive supranuclear palsy (PSP), and control subjects without known neurodegenerative condition.

Background: The identification of mutations of the COQ2 gene (whose protein product is pivotal for the biosynthesis of coenzyme Q10) in familial and sporadic MSA and the subsequent finding of reduced coenzyme Q10 levels in the cerebellum of MSA brains, suggest that coenzyme Q10 is relevant to MSA pathophysiology and that it might be both a biomarker and a potential therapeutic target for MSA. Two recent studies have shown reduced coenzyme Q10 levels in serum and plasma (respectively) of MSA patients. However, in the serum study differences were significant only when controlling for cholesterol levels (a known confounder of blood coenzyme Q10 levels), whereas the plasma study included MSA and controls, but not other parkinsonisms, with remarkably overlapping coenzyme Q10 values. In such scenario, cerebrospinal fluid (CSF) might be more specific to assess coenzyme Q10 levels within the central nervous system.

Methods: Convenience cohort study of CSF from 20 MSA patients from the Biorepository of the Catalan MSA Registry (CMSAR), along with CSF from 15 PD, 10 PSP and 15 controls available at our Movement Disorders Unit Biospecimen Collection. A commercial ELISA kit specific for coenzyme Q10 was used. The study received Ethics approval and all participants provided their written informed consent.

Results: All study groups were comparable in terms of sex, whereas PD and controls were significantly older than MSA and PSP participants. CSF levels of coenzyme Q10 were significantly lower in MSA vs. PD, PSP, and controls. The association remained significant in binary logistic regression models adjusted for both sex and age in MSA vs. PD and MSA vs. PSP. Discriminant ROC analysis yielded significant AUCs for MSA vs. PD, MSA vs. PSP and MSA vs. controls (all AUCs >0.80; all p-values <0.005). CSF coenzyme Q10 levels did not differ between MSAp and MSAc (n= 10 each).

Conclusions: With the limitations of the uneven age distribution and the lack of a validation cohort, the current findings indicate that low CSF coenzyme Q10 levels deserve further consideration as a potential biomarker of MSA. The role of age as a potential confounder will need to be established. This study and the CMSAR are funded by “Fundació La Marató de TV3”.

References: 1.- Kasai T, Tokuda T, Ohmichi T, Ishii R, Tatebe H, Nakagawa M, Mizuno T. Serum Levels of Coenzyme Q10 in Patients with Multiple System Atrophy. PLoS One 2016; 11: e0147574. 

2.- Mitsui J, Matsukawa T, Yasuda T, Ishiura H, Tsuji S. Plasma Coenzyme Q10 Levels in Patients With Multiple System Atrophy. JAMA Neurol 2016; 73: 977-80.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/cerebrospinal-fluid-levels-of-coenzyme-q10-are-reduced-in-multiple-system-atrophy/