We confirmed the upregulations of LC3-II and insoluble p62 in GM1 gangliosidosis-derived skin fibroblasts compared with normal controls, suggesting that autophagy initiation is upregulated in the fibroblasts, in turn impairing autophagic flux; however, both markers were not at all enhanced in GM1 gangliosidosis-derived NSCs. Here, SQSTM1 is linked to GM1 gangliosidosis.