Conference Coverage
Patients with multiple system atrophy (MSA) have decreased levels of plasma coenzyme Q10 (CoQ10), regardless of their COQ2 genotype, according to a study published in the August issue of JAMA Neurology. This finding, researchers said, supports the hypothesis that supplementation with CoQ10 is beneficial for patients with MSA.
It has been reported that functionally impaired variants of COQ2, which encodes an essential enzyme in the biosynthetic pathway of CoQ10, are associated with MSA. However, little is known about blood levels of CoQ10 in patients with or without COQ2 mutations or about the role of CoQ10 in the pathogenesis of MSA.
To explore whether there are associations between levels of CoQ10 in the blood and MSA, Jun Mitsui, MD, PhD, a neurogeneticist at the University of Tokyo, and colleagues compared the levels of plasma CoQ10 in patients with MSA with those in age-, sex-, and COQ2 genotype-matched controls.
The study included 44 Japanese patients with MSA (mean age, 63.7) and, for comparison, 39 Japanese control patients (mean age, 60.3).
The authors reported that the mean plasma level of CoQ10 in patients with MSA was lower than that in controls (0.51 μg/mL vs 0.72 μg/mL). The mean plasma levels of CoQ10 in patients with the cerebellar variant of MSA and those with the parkinsonian variant of MSA were 0.58 μg/mL and 0.49 μg/mL, respectively. After adjusting for age, sex, and COQ2 genotype, the plasma levels of CoQ10 were significantly associated with MSA.
“Prospective cohort studies are warranted to determine the longitudinal effects of plasma levels of CoQ10 on the development of MSA. Furthermore, future clinical trials of supplementation with CoQ10 in patients with MSA are required to confirm our hypothesis,” Dr. Mitsui and colleagues said.
In an accompanying editorial, Sheng-Han Kuo, MD, and Catarina M. Quinzii, MD, both of Columbia University in New York, said, “The recent discoveries of COQ2 mutations in rare patients with MSA and of CoQ10 deficiency in the brain and blood of patients with MSA may lead to mechanism-based and rational therapies for MSA, a rapidly progressive disorder in need of a disease-modifying therapy.”
—Glenn S. Williams