TDP-43 overexpression in APP/PSEN1 mice decreased Aβ plaque deposition but increased Tau aggregation (Davis et al, 2017), and cytoplasmic accumulation of endogenous phosphorylated TDP-43 was reported in two different Tau transgenic mouse models, but not in mouse models of Aβ deposition, α-synucleinopathy or Huntington’s disease (Clippinger et al, 2013). Here, TARDBP is linked to juvenile Huntington disease.