Recently, a novel TDP-43 mouse model of ALS expressing human transgene cDNA under the control of the murine prion promoter (mPrP) was created on a mixed C57BL/6;CBA genetic background (Borchelt and Sisodia, 1996; Wegorzewska et al., 2009). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.