Mutations in other nuclear genes such as Sco1 (Mourier et al., 2014), Sco2 (Joost et al., 2010), Cox10 (Antonicka et al., 2003), Cox15 (Mourier et al., 2014), LRPPRC (Rolland et al., 2013; Mourier et al., 2014), TACO1 (Weraarpachai et al., 2009), PET100 (Lim et al., 2014), C12orf65 (Wesolowska et al., 2015), which play important roles in COX assembly and biogenesis, have all been implicated in LS pathology. Here, LRPPRC is linked to Leigh syndrome.