Homozygous Surf1 mutant mice with cytochrome c oxidase deficiency (Agostino et al., 2003), mice lacking mitochondrial superoxide dismutase (Melov et al., 1998), and mice deficient in the mitochondrial membrane protease Presenilins-associated rhomboid-like protein (PARL) (Spinazzi et al., 2019) have all been created to model LS. Here, SURF1 is linked to Leigh syndrome.