Previous studies using cells from LS patients carrying homozygous SURF1 (c.769G>A and c.530T>G) and MT-ATP6 (m.9185T>C) mutations showed an abnormal generation of neural lineages (Lorenz et al., 2017) and impaired neurogenesis in cerebral organoids (Inak et al., 2021). This evidence concerns the gene MT-ATP6 and Leigh syndrome.