Bacterial artificial chromosome (BAC) transgenic mice mainly reproduce RNA foci formation and DPR inclusions, but only rare or unstable TDP-43 pathology, neurodegeneration or FTD/ALS phenotype (O’Rourke et al., 2015; Peters et al., 2015; Jiang et al., 2016; Liu et al., 2016; Mordes et al., 2020; Nguyen et al., 2020). Here, TARDBP is linked to amyotrophic lateral sclerosis.