Coenzyme Q (CoQ) is an essential lipid component of the mitochondrial electron transport chain and is required for optimal ATP production. Mutations in genes of the CoQ biosynthetic pathway cause rare but severe diseases with symptoms ranging from deafness and myopathy to progressive neurodegeneration. In addition, it is thought that dietary supplementation with CoQ decreases the pathology of some neurodegenerative disorders, such as Parkinson’s disease. Grant et al. describe a Drosophila model of primary CoQ deficiency in the developing CNS that might help to optimise treatments for primary CoQ deficiency diseases in humans, as well as to investigate the efficacy and mechanism of action of over-the-counter CoQ dietary supplements.
2010
