Since activation of the BNIP3/NIX pathway is known to induce cell death [51], we may speculate that due to the heightened severity of mitochondrial dysfunction in Alpers’ syndrome, BNIP3 may be upregulated both to induce mitophagy, and may contribute to the activation of cell death pathways preceding neuronal attrition. Here, BNIP3 is linked to Alpers syndrome.