PRNP and prion disease: Pathogenic PRNP mutations appear to have diverse and direct effects on dictating the preferred structure or assembly state of mutant PrP prion assemblies and cases of inherited prion disease caused by point mutations have PrP 27–30 glycoform ratios distinct from those seen in both sporadic and acquired CJD human prion diseases (Piccardo et al. 1998; Parchi et al. 1998; Furukawa et al. 1998; Cardone et al. 1999; Hill et al. 2006).