Mutations in conserved residues of Cox10, Cox15 and SURF1 manifest in tubulopathy and leukodystrophy [158], sensorineural deafness [159], fatal infantile hypertrophic cardiomyopathy [159,160,161], Charcot-Marie-Tooth disease type 1A [162] and neurologic LS [153,159,163,164,165,166,167,168,169,170]. The gene discussed is SURF1; the disease is Leigh syndrome.