In this study, we aim to establish new sialidosis-specific neural models by producing sialidosis-specific iPSCs and NPCs with NEU1 mutations (p.V275A/R347Q and p.G227R) to investigate the effects of NEU1 deficiency on autophagy–lysosome pathways and their relevant contribution to neurological defects. The gene discussed is NEU1; the disease is sialidosis.