NDUFA12 impairment is strongly associated with Leigh syndrome in humans, a paediatric mitochondrial disorder characterized by brain-specific anomalies and early death (Magrinelli et al. 2022; Ostergaard et al. 2011; Rak and Rustin 2014; Torraco et al. 2021) for which no fully compensatory mechanisms are known (Adjobo-Hermans et al. 2020; van de Wal et al. 2022). This evidence concerns the gene NDUFA12 and Leigh syndrome.