Table 1.

Spontaneous neurologic illness in Tg mice expressing mutant BVPrP(I109)

Mutation	Line	Transgene copy numbera	Relative Prnp mRNA level (-fold) ± SEMb	Relative PrP expression level (-fold)c	Mean age of disease onset ± SEM (d)	Signs of neurologic dysfunction (n/n0)d
wt	Tg3574	17	1.0 ± 0.3	3.6e	340 ± 26e	14/14e
	Tg3581	25	1.5 ± 0.7	5.3e	218 ± 9e	19/19e
D178N	Tg15972	20	1.6 ± 0.4	0.4	240 ± 11	24/24
	Tg15464	NDf	NDf	0.4	221 ± 9	23/23
	Tg15465	NDf	NDf	0.4	197 ± 8	22/22
	Tg15965	33	1.6 ± 0.2	0.5	179 ± 8	22/22
E200K	Tg14205	NDf	NDf	0.6	555 ± 29	12/12
	Tg14210	8	0.9 ± 0.1	1.2	460 ± 31	12/12
	Tg7253	17	1.8 ± 0.3	1.7	268 ± 16	24/24
	Tg4253	31	2.8 ± 0.4	2.4	162 ± 7	24/24
	Tg7271	50	2.1 ± 0.4	2.7	119 ± 2	24/24
ΔGPI	Tg24600	22	1.6 ± 0.2	0.5	415 ± 15	11/11

^aTransgene copy numbers were determined by qPCR on genomic DNA samples using Syrian hamster DNA as a control.

^bPrnp mRNA levels were determined by qPCR on brain cDNA samples and then normalized to Prnp mRNA levels in Tg3574 mice.

^cRelative PrP expression levels were determined by ELISA relative to expression of wt MoPrP in FVB mouse brain.

^dn, number of positive mice; n₀, number of examined mice.

^eData previously reported in Ref. [64].

^fND, not determined.