In a mouse model of the polyglutamine disease, dentatorubral-pallidoluysian atrophy (DRPLA) as well as human fibroblasts of DRPLA patients, pathological manifestations include inhibition of LC3 lipidation, p62 accumulation and reduced transcription factor EB (TFEB) expression, rendering autophagy a stalled mechanism (Baron et al., 2017). The gene discussed is SQSTM1; the disease is Dentatorubral pallidoluysian atrophy.