Human iPSC lines were obtained from the Target ALS Repository, Harvard University, Jackson Laboratory, and the NINDS Human Cell and Data Repository (NHCDR). Human iPSC lines included non-isogenic control lines 11a [9 (link)], ND50003 (FA0000010) and KOLF2.1 J [75 (link)] and five fALS lines harboring mutations in C9orf72: 19f [45 (link)], NDS00268 (ND50074), NDS00269 (ND50075), NDS00270 (ND50076), and NDS00273 (ND50080). An isogenic pair of lines consisting of an unedited control TDP-43+/+ line and an edited TDP-43+/G298S iPSC line (harboring a single fALS mutation) was previously generated [77 (link)]. iPSCs were cultured as described previously [65 (link)]. Briefly, iPSCs were maintained on Matrigel-coated 6 well-plates (Corning, 354,277, and Falcon, 353,046) in mTeSR media (Stemcell Technologies, 85,850) for SMN differentiation or mTeSR Plus media (Stemcell Technologies, 100–0274) for NGN2 induction at 37 °C with 5% CO2.
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