Intriguingly, the disparities in OXPHOS protein deficiencies between the Alpers’ syndrome and adult PMD patient groups were mirrored by an increased expression of the autophagy- and mitophagy-related proteins LC3B and BNIP3 within Purkinje cells of patients with Alpers’ syndrome. The gene discussed is MAP1LC3B; the disease is Pelizeaus-Merzbacher spectrum disorder.