In a mouse model of dentatorubral-pallidoluysian atrophy (DRPLA), a polyglutamine repeat-associated ataxia, canonical autophagy is inhibited, while nucleophagy-based Lamin B1 degradation and Golgi membrane-associated excretion is activated [89]. Here, LMNB1 is linked to dentatorubral-pallidoluysian atrophy.