Table 1.

Influence of the codon 129 polymorphism on the susceptibility to sCJD(MM1) and sCJD(VV2) prions in transgenic mice^a

Mouse line	Expression level	Uninoculated		sCJD(MM1) prions		sCJD(VV2) prions
		No. of days (95% CI) until spontaneous disease	n/n0	Incubation period, days (95% CI)	n/n0	Incubation period, days (95% CI)	n/n0
Tg(HuPrP,M129)440b	2	>600	0/9	162 (159, 170)	33/33	>600	6/21e
Tg(MHu#2,M129)22372b	0.8	>600	0/12	111 (107, 112)	49/49	>600	1/19f
Tg(MHu#2,M129)17103	1.2	>600	0/6	103 (99, 104)	7/7	ND
Tg(MHu#2,M129+/+)22372c	1.6	>600	0/17	124 (123, 126)	34/34	ND
Tg(MHu#2,M129)17051	2.8	>600	0/7	167 (165, 194)	8/8	ND
Tg(MHu#2,M129)17062	4.9	289 (226, 312)	6/6	244 (235, 255)d	18/18	ND
Tg(HuPrP,V129+/+)152	4	>600	0/7	211 (193, 214)	7/7	193 (167, 208)	8/8
Tg(MHu#2,V129)10355	1.1	>600	0/7	125 (117, 148)	8/8	>600	0/6
Tg(MHu#2,V129)7104	1.3	>600	0/7	133 (130, 134)	8/8	>600	0/7
Tg(MHu#2,V129)6550	1.8	>600	0/7	209 (193, 227)	8/8	>600	1/5g
Tg(MHu#2,V129)7110	2.4	>600	0/7	207 (196, 214)	8/8	>600	4/16h

^aPrP expression level in brain relative to FVB mice, reported as mean fold expression from two to four brains. Spontaneous disease from time of birth and incubation periods from time of inoculation are reported as median time to onset of clinical signs in days, with 95% confidence intervals, calculated using Kaplan-Meier statistics. n, number of mice with clinical signs of disease; n_0, number of mice monitored; ND, not determined.

^bIncludes data previously reported in reference 20.

^cIncludes data previously reported in reference 28.

^dWhen recalculated from date of birth, durations not significantly different from spontaneous disease in uninoculated mice.

^eIndividual mice showed clinical disease at 329, 336, 339, 339, 482, and 535 dpi.

^fOne mouse showed clinical disease at 449 dpi.

^gOne mouse showed clinical disease at 476 dpi.

^hIndividual mice showed clinical disease at 473, 494, 509, and 540 dpi.