In cerebellar tissues from patients with Alpers’ syndrome, remaining Purkinje cells demonstrated a marked increased expression of both BNIP3 and LC3B proteins, compared to controls and patients with late-POLG and mtDNA disease (Fig. 4), mirroring the more extensive and severe OXPHOS deficiencies also observed in this patient group. The gene discussed is POLG; the disease is Alpers syndrome.