TABLE 3.
Isolate |
tgMet |
tgMet/Val |
tgVal |
||||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Passage 1 |
Passage 2 |
Passage 1 |
Passage 2 |
Passage 1 |
Passage 2 |
||||||||||||||||
Case | Codon 129 |
PrPres
type |
Brain areab |
D/Ic | IncubPd | PrPres
type |
D/I | IncubP | PrPres
type |
D/I | IncubP | PrPres
type |
D/I | IncubP | PrPres
type |
D/I | IncubP | PrPres
type |
D/I | IncubP | PrPres
type |
21 | VV | 1 | Caudate | 6/6 | 346 ± 75 | 1 | 6/6 | 215 ± 12 | 1 | 6/6 | 369 ± 52 | 1 | 6/6 | 245 ± 8 | 1 | 6/6 | 196 ± 7 | 2 | 6/6 | 174 ± 7 | 2 |
2 | Cerebellum | 5/5 | 569 ± 89 | 1 | 6/6 | 504 ± 74 | 1 | 6/6 | 483 ± 23 | 1 | 6/6 | 504 ± 54 | 1 | 6/6 | 184 ± 11 | 2 | 6/6 | 170 ± 10 | 2 | ||
2 | Temp. Cort | 6/6 | 333 ± 61 | 1 | 6/6 | 221 ± 8 | 1 | 6/6 | 385 ± 61 | 1 | 6/6 | 241 ± 10 | 1 | 6/6 | 208 ± 8 | 2 | 6/6 | 170 ± 9 | 2 | ||
22 | MV | 2 | Caudate | 6/6 | 518 ± 84 | 1 | 6/6 | 571 ± 14 | 1 | 6/6 | 466 ± 90 | 1 | 5/5 | 476 ± 26 | 1 | 6/6 | 180 ± 8 | 2 | 6/6 | 161 ± 8 | 2 |
1+2 | Cerebellum | 6/6 | 357 ± 38 | 1 | 6/6 | 220 ± 5 | 1 | 6/6 | 396 ± 66 | 1 | 6/6 | 261 ± 11 | 1 | 6/6 | 180 ± 4 | 2 | 6/6 | 175 ± 5 | 2 | ||
2 | Occip. Cort | 6/6 | 277 ± 50 | 1 | 6/6 | 210 ± 5 | 1 | 6/6 | 387 ± 17 | 1 | 6/6 | 245 ± 21 | 1 | 6/6 | 217 ± 23 | 2 | 6/6 | 164 ± 7 | 2 | ||
23 | MV | 1+2 | Caudate | 5/6 | 519 ± 88 | 1 | 6/6 | 212 ± 8 | 1 | 6/6 | 523 ± 82 | 1 | 6/6 | 250 ± 2 | 1 | 6/6 | 251 ± 66 | 2 | 6/6 | 168 ± 3 | 2 |
2 | Cerebellum | 0/5 | >750* | 1 | 6/6 | 579 ± 20 | 1 | 0/6 | >750* | 1 | 6/6 | 484 ± 40 | 1 | 6/6 | 316 ± 52 | 2 | 6/6 | 166 ± 4 | 2 | ||
1 | Temp. Cort | 1/6 | 689, 718* | 1 | 6/6 | 254 ± 30 | 1 | 0/6 | >750 | −e | ND | 6/6 | 362 ± 58 | 1 | 6/6 | 193 ± 13 | 2 |
Three sCJD cases displaying different PRNP genotypes at codon 129 (VV, homozygous Val129; MV, heterozygous Met/Val129) and showing mixed type 1/type 2 PrPres Western blot profiles in either the same or different brain areas were selected. Three brain areas from each case were inoculated (two iterative passages) in transgenic mice that express the Met129 (tgMet) or Val129 (tgVal) human PrP and their crossbreed (tgMet/Val) were inoculated intracerebrally (20 μl per mouse). Brains from first passage mice (PrPres positive in the brain) were pooled and used for the second passage. The PrPres WB isoforms (type 1 or type 2) identified in mouse brains are reported.
Abbreviations: Caudate, caudate nucleus; Temp. Cort, temporal cortex; Occip. Cort, occipital cortex.
D/I, number of diseased mice/number of inoculated mice. *, PrPres positive in a found dead animal without TSE clinical signs.
IncubP, incubation period (time to death [in days]) shown as means ± standard deviations. An asterisk (e.g., >750*) indicates that all mice were PrPres positive, but showed no clinical signs when killed (end of their life span). ND, not done.
−, PrPres negative.