EPG5 and hyperinsulinemic hypoglycemia, familial, 4: 2) Animal models of defective autophagy show CNS features mimicking adult-onset neurodegenerative disorders, as demonstrated in a conditional Epg5 Drosophila knockdown showing severe retinal neurodegeneration [84], by clinical and pathological features of human ALS in the epg5−/- mouse [129], and by similar findings in a range of murine models of Atg deficiency [225].