Third, LS‐encephalomyopathy showed a substantial loss of neurons, increased neuronal apoptosis and increased infiltration of the cerebellum and brainstem by activated microglia, concomitant with an increase in acetylated‐p53 and overall increased inflammatory phenotype reported in Ndufs4 deficient mice.29 Here, NDUFS4 is linked to mitochondrial DNA depletion syndrome, encephalomyopathic form with methylmalonic aciduria.