To examine inflammation triggered by TDP-43, we employed inducible expression of empty vector (control [Ctrl]), wild-type (WT), or ALS mutant (Q331K) TDP-43 in a mouse neuronal cell line, which confirmed upregulation of NF-κB and type I IFN pathways in vitro (Figures S1A–S1C). Here, TARDBP is linked to amyotrophic lateral sclerosis.