It is also worth noting that TDP-43A382T and TDP-43G348C MNs did not mirror the transcriptional signature of TDP-43 loss-of-function (implicating STMN2, UNC13A, ELAVL3, PFKP, RCAN1, SELPLG) established by previous knockdown studies and other ALS iPSC models16,17,51,63,64,81,82, perhaps consistent with the preserved nuclear localization of TDP-43 in our cells44. Here, TARDBP is linked to amyotrophic lateral sclerosis.