A 2-year longitudinal study of swimming navigation in mice devoid of the prion protein: no evidence for neurological anomalies or spatial learning impairments.

Uncontrolled accumulation of a conformationally distorted protein (PrP(Sc)) is supposed to be the pathological process leading to spongiform encephalopathy. Targeted disruptions of the Prn-P gene in the mouse have resulted in animals that did not show anomalies in spatial and avoidance learning and were resistant to experimental infections. However, another Prn-P knockout mouse was reported to show ataxia and Purkinje cell degeneration developing after 70 weeks of age.