A full-length human TAR DNA binding protein (TARDBP or TDP-43) cDNA sequence was modified to have an N-terminal myc tag. This huTDP-43-WT cDNA sequence was inserted between exon 2 and exon 3 of mouse prion protein (Prnp) gene. The resulting Prnp-TDP-43-WT transgene was flanked with loxP sites. Three lines (65 93 and 96) were produced. Founder line 96 was identified with about 1.5-fold greater total TDP-43 expression levels in spinal cord as compared to endogenous TDP-43 in non-transgenic mice.