To gain deeper insights into the impact of autophagic defects on MN degeneration in the context of C9-ALS/FTD, we used patient-derived hiPSC-derivedMNs and CRISPR/Cas9-mediated genome editing technology to create a C9orf72 knockout hiPSC line, allowing us to assess the functional consequences of reduced C9orf72 protein levels on the autophagy-lysosome pathway. Here, C9 is linked to amyotrophic lateral sclerosis.