| Table 6. Distinct prion strains generated in humans
with inherited prion diseases and transmitted to transgenic (Tg) mice |
|
| Inoculum |
Host species |
Host PrP genotype |
Incubation time, days |
(n/n0) |
PrPSc, kDa |
|
| None |
Human |
FFI(D178N,
M129) |
--- |
--- |
19 |
| FFI |
Mouse |
Tg(MHu2M) |
206 ± 7 |
(7/7) |
19 |
| FFI passaged
through Tg(MHu2M) |
Mouse |
Tg(MHu2M) |
136 ±
1 |
(6/6) |
19 |
| None |
Human |
fCJD(E200K) |
|
|
21 |
| fCJD |
Mouse |
Tg(MHu2M) |
170 ±
2 |
(10/10) |
21 |
| fCJD passaged through Tg(MHu2M) |
Mouse |
Tg(MHu2M) |
167 ± 3 |
(15/15) |
21 |
|
| Incubation times are given ±SEM. |
|