These samples harboured ubiquinated TDP-43 cytoplasmic inclusions and were from patients with no mutations in known ALS-causative genes, including FUS, TDP-43 or TAF15. Intriguingly, our comparisons of RNA signatures revealed an inverse correlation in a separate set of genes that were upregulated in the sALS samples but were downregulated upon loss of ALS-associated RBPs in in vitro-derived MNs. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.