The Aspanur7/nur7 mouse contains a nonsense mutation (Q193X) in the ASPA gene.[17] Because Aspanur7/nur7 mice exhibit key pathological phenotypes resembling those of CD patients, including loss of ASPA enzymatic activity, elevated NAA levels, and extensive spongy degeneration in various brain regions,[17] it is considered a relevant animal model for CD. This evidence concerns the gene ASPA and Cowden disease.