NDUFS8 and Leigh syndrome: The ND-2360114 and mt:ND2del1 lines of fruit flies (Drosophila melanogaster) are models of LS caused by hypomorphic mutations in the nuclearly encoded ND-23 (mammalian Ndufs8) and the mitochondrially encoded mt:ND2 (mammalian ND2) subunits that are conserved proteins of the core of Complex I. They mimic the age-dependent neurodegeneration, mitochondrial abnormalities and shortened lifespan of LS patients [8,10] as well as hypersensitivity to VGAs [4,11].