Our Prnp92N model builds on important findings revealed from these noninfectious N-terminal mutation models, however, differs in being a knockin mouse expressing full-length PrPC with 1 residue substitution (more than 99% of sequence conserved) that more closely resembles the clinical and pathologic phenotype of infectious prion disease models and is suggestive of heightened neuronal activity. This evidence concerns the gene PRNP and prion disease.