In summary, these data suggest that β-RA therapeutic effects in Coq7/COQ7 and Coq9/COQ9 dysfunction are mediated by decreased DMQ levels; however, additional mechanisms must be involved, as β-RA prevents the encephalopathy in Coq9 mutant mice without affecting CoQ9 and DMQ9 levels in these mice’s brains [5,61], and it recovered the pathogenic phenotype in the Coq6 and Coq8b mouse models. Here, COQ9 is linked to Encephalopathy.