β-RA reduces DMQ/CoQ ratio and recues the encephalopathic phenotype in Coq9R239X mice
Coenzyme Q (CoQ) deficiency has been associated with primary defects in the CoQ biosynthetic pathway or to secondary events. In some cases, the exogenous CoQ supplementation has limited efficacy. In the Coq9R239X mouse model with fatal mitochondrial encephalopathy due to CoQ deficiency, we have tested the therapeutic potential of b-resorcylic acid (b-RA), a structural analog of the CoQ precursor 4-hydroxybenzoic ... Read more