The transgenic construct contains the Tet-responsive element and mouse prion protein non-coding exons 1-2 upstream of a cDNA sequence encoding amino acids 208-414 of human TAR DNA binding protein (TARDBP or TDP-43) followed by the mouse prion protein non-coding exon 3 and 3' UTR of the moPrP.XhoI vector (including polyadenylation signal). Three founder lines were generated. Founder line 2 had the highest inducible expression of 208 TDP-43 C-terminal fragments (CTFs), and all three lines have the same inducible 208 TDP-43 CTF distribution and biochemical properties.