The construct contains the full-length human TAR DNA binding protein (TARDBP or TDP-43) cDNA sequence that was modified to harbor an N-terminal Flag tag immediately after the start methionine and the A315T amino acid substitution associated with familial ALS inserted between exon 2 and exon 3 of mouse prion protein (PrP or Prnp) gene. A single Prp-TDP43A315T founder line was established (founder line 95) mapping to two close but distinct sites on Chr 9: Chr9:38,417,354 and Chr9:38,405,898 (coordinates from MGSC ver 37, mm9). (J:153197)