Some years ago, in an effort to model NIIDs and CIPO, Clarke et al. showed that a transgenic (Tg) mouse model for SCA7, the PrP-SCA7-92Q line that expresses human ataxin-7 with an expanded polyQ tract under the prion protein promoter, could be a suitable model for GI neuropathy, as seen in NIID patients (Clarke et al., 2007). Here, PRNP is linked to chronic intestinal pseudoobstruction.