Table 2. Transmission of human prions to transgenic mice heterozygous for human PrP V127.
| Inoculum |
Transmission data |
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|---|---|---|---|---|---|---|---|---|
| Aetiology | Source Code | Human PrPSc type* | Attack rate† | Incubation period, (days ± SEM) or (days p.i) | Attack rate† | Incubation period, (days ± SEM) or (days p.i) | Attack rate† | Incubation period, (days ± SEM) or (days p.i) |
|
| ||||||||
| A | Tg35c G127M129/G127M129 |
Tg35c × Tg183 G127M129/V127M129 |
Tg35c × Tg190 G127M129/V127M129 |
|||||
| kuru | I516 | T3 VV | 14/14 | 509 ± 56 (3) | 0/11 | >518-605 | 0/13 | >517-605 |
| kuru | I520 | T3 VV | 12/12 | 558 ± 4 (4) | 0/14 | >525-622 | 0/14 | >460-622 |
| kuru | I10336 | T3 MV | 13/13 | 454 ± 14 (10) | 0/14 | >522-606 | 0/11 | >504-607 |
| kuru | I518 | T2 MM | 11/11 | 493 ± 32 (7) | 0/12 | >545-620 | 0/13 | >544-603 |
| iCJD (GH) | I035 | T1 MM | 10/10 | 221 ± 3 (10) | 0/10 | >462-603 | 0/8 | >506-602 |
| sCJD | I11058 | T1 MM | 8/8 | 231 ± 3 (8) | 0/8 | >467-605 | 0/7 | >487-605 |
| iCJD (DM) | I026 | T2 MM | 10/10 | 256 ± 9 (8) | 0/8 | >507-601 | 0/7 | >520-602 |
| sCJD | I7040 | T2 MM | 10/10 | 233 ± 3 (10) | 0/8 | >582-604 | 0/7 | >600-604 |
| B | Tg152c G127V129/G127V129 |
Tg152c × Tg183 G127V129/V127M129 |
Tg152c × Tg190 G127V129/V127M129 |
|||||
|---|---|---|---|---|---|---|---|---|
| kuru | I516 | T3 VV | 9/9 | 218 ± 1 (6) | 0/13 | >447-608 | 9/15 | 543, 592 |
| kuru | I520 | T3 VV | 9/9 | 196 ± 7 (7) | 0/18 | >491-616 | 13/13 | 498 ± 17 (5) |
| kuru | I10336 | T3 MV | 15/15 | 212 ± 3 (11) | 15/15 | 456 ± 3 (15) | 15/15 | 316 ± 4 (14) |
| kuru | I518 | T2 MM | 11/11 | 211 ± 4 (8) | 3/11 | >494-620 | 13/14 | 468 ± 13 (9) |
| sCJD | I280 | T2 VV | 8/8 | 203 ± 5 (4) | 0/4 | >600-602 | 10/10 | 559 ± 14 (8) |
| sCJD | I278 | T2 VV | 6/6 | 236 ± 8 (6) | 0/8 | >424-602 | 8/9 | 582 |
| sCJD | I284 | T2 MV | 9/9 | 338 ± 4 (8) | 0/9 | >410-602 | 5/7 | >545-608 |
| sCJD | I1478 | T2 MV | 10/10 | 248 ± 8 (8) | 0/6 | >451-608 | 2/7 | >482-603 |
| sCJD | I7394 | T3 VV | 9/9 | 213 ± 1 (8) | 7/7 | >537-677 | 10/10 | 427 ± 8 (10) |
| sCJD | I764 | T3 MV | 10/10 | 219 ± 5 (7) | 9/10 | >368-588 | 10/10 | 461 ± 7 (9) |
| iCJD (GH) | I2651 | T3 VV | 8/8 | 200 ± 3 (6) | 0/6 | >567-607 | 7/7 | 538 ± 2 (5) |
| iCJD (GH) | I020 | T3 MV | 9/9 | 211 ± 2 (6) | 7/7 | >489-602 | 10/10 | 399 ± 8 (9) |
| C | Tg152c × Tg183 G127V129/V127M129 |
Tg152c × Tg190 G127V129/V127M129 |
Tg152c+/o (Hemi) G127V129 |
|||||
|---|---|---|---|---|---|---|---|---|
| iCJD (GH) | I020 | T3 MV | 7/7 | >489-602 | 10/10 | 399 ± 8‡ (9) | 14/14 | 252 ± 3‡ (14) |
| sCJD | I7394 | T3 VV | 7/7 | >537-677 | 10/10 | 427 ± 8‡ (10) | 12/12 | 245 ± 5‡ (12) |
| kuru | I10336 | T3 MV | 15/15 | 456 ± 3 (15) | 15/15 | 316 ± 4‡ (14) | 15/15 | 222 ± 2‡ (15) |
| D | Tg35c G127M129/G127M129 |
Tg35c × Tg183 G127M129/V127M129 |
Tg35c × Tg190 G127M129/V127M129 |
|||||
|---|---|---|---|---|---|---|---|---|
| vCJD | I342 | T4 MM | 15/15 | >426-603 | 9/15 | 467, 556 | 17/18 | 419±17 (3) |
| vCJD | I7042 | T4 MM | 14/14 | 559, 561 | 4/13 | >496-607 | 10/12 | 596 |
iCJD, iatrogenic CJD; sCJD, sporadic CJD; GH, growth hormone; DM, dura mater
According to classification of Hill et al.28
Attack rate is defined as the total number of both clinically affected and sub-clinically infected mice as a proportion of the total number of inoculated mice. Sub-clinical prion infection was assessed by immunoblotting and/or immunohistochemical examination of brain. Incubation periods are reported for clinically affected mice in days; where n ≥ 3 the mean ± SEM is reported with the number of mice contributing to the mean shown in parentheses, otherwise individual incubation times are given. In groups where no clinical transmission of prion disease was observed, the attack rate represents subclinical infection only and the interval between inoculation and death (from either senescence, culling due to inter-current illness or termination of the experiment) is reported as >x-y days.
Mean incubation periods for all 3 isolates are significantly lower in Tg152c hemizygotes than in Tg190 × Tg152c heterozygotes (P<0.0001; two-tailed unpaired t-test).