Mouse embryonic fibroblasts (MEFs) lacking C9ORF72 and C9ALS/FTD patient-derived iPS-derived neurons displayed reduced macroautophagy initiation, accumulation of SQSTM1/p62, an adaptor and substrate for macroautophagy degradation, and TDP-43 (Sellier et al., 2016; Webster et al., 2016), protein hallmarks of ALS/FTD affected brains. Here, TARDBP is linked to amyotrophic lateral sclerosis.