Some ALS- and FTD-associated TBK1 variants have previously been shown to diminish or abolish phosphorylation of the TBK1 target IRF3 (Freischmidt et al., 2015, Kim et al., 2016, Pozzi et al., 2017, Tsai et al., 2016). Here, TBK1 is linked to frontotemporal dementia.