Table 3.

Effects of various mutations in the post-OR region of PrP^C on hereditary and acquired prion diseases.

Disease Type	PrPs	The Post-OR Sequence	Clinicopathological Features	References
Hereditary prion disease	PrP-P101L	Proline residue at position 101 mutated to leucine residue in mouse PrP	• Spontaneously develop prion disease-like diseases. • Accumulate weakly protease-resistant PrP-P101L in the brain. • Accumulate prion infectivity associated with weakly protease-resistant PrP-P101L.	[77,78]
	PrP-A116V	Alanine residue at position 116 mutated to valine residue in mouse PrP	• Spontaneously developed prion disease-like diseases. • Accumulate partly insoluble and weakly protease-resistant PrP-A116V in the brain. • No data available as to infectivity associated with protease-resistant PrP-A116V.	[79]
Disease Type	PrPs	The Post-OR Sequence	Susceptibility to Prions	References
Acquired prion disease	PrP∆32–80	Intact	• Fully susceptible to RML scrapie prions.	[80]
	PrP∆32–93	The post-OR residues 91–93 deleted	• Partially reduced to RML scrapie prions.	[81]
	PrP∆32–106	The post-OR residues 91–106 deleted	• Resistant to RML scrapie prions.	[82]