SURF1 and Leigh syndrome: SURF1, encoding a factor involved in the biogenesis of cytochrome c oxidase, is associated with the Leigh syndrome, a mitochondrial disorder caused by mutations of SURF1. In 2007, D’Agnello et al. [71] showed that adult SURF1−/− mice were protected from neurodegeneration at any age, showing prolonged lifespan and complete protection from Ca2+-dependent neurotoxicity induced by kainic acid [71].