In this study, we used iPSC-derived MNs harbouring mutations in two different genes associated with ALS, TARDBP and C9ORF72. TARDBP encodes the RNA-binding protein TDP-43, which can act as a transcription repressor and a splicing regulator, and can also contribute to RNA stability and transport50, 51, 52. Here, C9orf72 is linked to amyotrophic lateral sclerosis.