Although conflicting results are available about the role of COQ2 mutations in MSA, a reduced CoQ10 amount has been observed in the MSA cerebellum (Barca et al., 2016; Schottlaender et al., 2016), cerebrospinal fluid (Compta et al., 2018), plasma (Mitsui and Tsuji, 2016), serum (Kasai et al., 2016), and fibroblasts (Monzio Compagnoni et al., 2018a), independently from COQ2 mutational status. The gene discussed is COQ2; the disease is multiple system atrophy.