Cytoplasmic accumulation of phosphorylated TDP-43 was specifically found in two tau transgenic models (P301L 0N4R-expressing rTg4510 mice and JNPL3 mice), but TDP-43 pathology was absent in mouse models of Aβ deposition (TgCRND8, Tg2576, and Tg2576 x P264L PS1 knock-in), α-synucleinopathy (A53T-expressing M83 mice and E46K-expressing M47 mice), or Huntington’s disease (N586-82Q-C63 model). Here, TARDBP is linked to Huntington disease.