Table 1.

Effects of various mutations in the polybasic region of PrP^C on acquired prion diseases.

Disease Type	PrPs	Amino Acid Sequence of the Polybasic Region (Residues 23–31) 1	Susceptibility to Prions	References
Acquired prion disease	WT PrP	KKRPKPGGW	• Normal.
	PrP∆23–31	− − − − − − − − −	• Markedly reduced to RML scrapie prions.	[63]
	PrP∆25–50	KK− − − − − − −	• Not reduced to RML and 22L scrapie prions	[64]
	PrP∆23–26	− − − −KPGGW	• Only slightly or not reduced to 127S and LA19K scrapie prions and BSE prions.	[65]
	PrP-M	KQHPHPGGW	• Markedly reduced to 127S and LA19K prions and BSE prions	[65]
	PrP3K3A	AARPAPGGW	• Markedly reduced to RML and 22L scrapie prions.	[66]

¹ Amino acids are indicated by single letters. Underline letters indicate amino acids mutated.