TABLE 2.
Effect of the P101L mutation on the generation and transmission of infectious prions
| Inoculuma,b | Recipientsb | PrPC expression levelc | Time to disease (days ± SEM) | n/n0d |
|---|---|---|---|---|
| None | Tg(MoPrP,P101L)196 | 1 | 552 ± 34 | 9/32 |
| None | Tg(MoPrP,P101L)2866 | 8 | 132 ± 2 | 19/19 |
| Tg(MoPrP,P101L)2866 | CD-1 | 1 | >700 | 0/8 |
| Tg(MoPrP,P101L)2866 | Tg(MoPrP-A)4053 | 8 | >700 | 0/7 |
| Tg(MoPrP,P101L)2866 | Tg(MoPrP,P101L)196 | 1 | 305 ± 17 | 10/10 |
| Tg(MoPrP,P101L)2866 | Tg(MoPrP,P101L)196 | 1 | 263 ± 12 | 8/8 |
| Tg196(2866) | Tg(MoPrP,P101L)196 | 1 | 351 ± 13 | 8/8 |
| Non-β-MoPrP(89-143,P101L)e | Tg(MoPrP,P101L)196 | 1 | 632 | 1/8 |
| β-MoPrP(89-143,P101L)e,f | Tg(MoPrP,P101L)196 | 1 | 360 ± 30 | 20/20 |
| Tg196(β-MoPrP(89-143,P101L))g | Tg(MoPrP,P101L)196 | 1 | 349 ± 8 | 8/8 |
| Tg196(β-MoPrP(89-143,P101L))g | Tg(MoPrP,P101L)196 | 1 | 351 ± 9 | 9/9 |
| Tg196(β-MoPrP(89-143,P101L))g | Tg(MoPrP,P101L)196 | 1 | 327 ± 9 | 8/8 |
| CD-1(RML) | CD-1 | 1 | 131 ± 0 | 29/29 |
| CD-1(RML)f | Tg(MoPrP,P101L)196 | 1 | 229 ± 6 | 6/6 |
| Tg196(RML)g | Tg(MoPrP,P101L)196 | 1 | 175 ± 1 | 7/7 |
| Tg196(RML)g | Tg(MoPrP,P101L)196 | 1 | 186 ± 3 | 9/9 |
| C57(139A) | CD-1 | 1 | 144 ± 2 | 14/14 |
| C57(139A)f | Tg(MoPrP,P101L)196 | 1 | 425 ± 4 | 9/9 |
| Tg196(139A)g | Tg(MoPrP,P101L)196 | 1 | 179 ± 8 | 9/9 |
| Tg196(139A)g | Tg(MoPrP,P101L)196 | 1 | 198 ± 6 | 9/9 |
| CD-1(301V) | CD-1 | 1 | 230 ± 3 | 10/10 |
| CD-1(301V) | B6.I | 1 | 132 ± 1 | 10/10 |
| B6.I(301V) | B6.I | 1 | 116 ± 1 | 17/17 |
| B6.I(301V) | CD-1 | 1 | 224 ± 5 | 8/8 |
| B6.I(301V)f | Tg(MoPrP,P101L)196 | 1 | 113 ± 4 | 9/9 |
| B6.I(301V)f | Tg(MoPrP,P101L)196 | 1 | 127 ± 2 | 3/3 |
| Tg196(301V)g | Tg(MoPrP,P101L)196 | 1 | 130 ± 3 | 5/5 |
| Tg196(301V)g | Tg(MoPrP,P101L)196 | 1 | 129 ± 1 | 4/4 |
Original prion inocula (RML, 139A, 301V) were obtained from Prnpa (C57, CD-1) and/or Prnpb mice (B6.I) or from spontaneously ill Tg(MoPrP,P101L)2866 mice. Homogenates used for transmissions originated from spontaneously ill or prion-inoculated mice at time of disease onset. The prion strain is shown in parentheses and the text preceding the parenthesis indicate the host in which it was last propagated.
All Tg(MoPrP,P101L) mice were Prnp0/0.
Levels of PrPC expression are compared with that of adult, wt FVB mice.
Number of ill mice (n) over the total number of mice under observation (n0).
Data from reference 29.
These inoculations correspond to the primary transmissions.
Inocula used for these secondary transmissions were prepared from brains obtained in the primary transmissions from spontaneously ill or prion-inoculated mice at time of disease onset.