Table 6.
Distinct prion strains generated in humans with inherited prion diseases and transmitted to 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 → 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 → Tg(MHu2M) | Mouse | Tg(MHu2M) | 167 ± 3 | (15/15) | 21 |
Data are from ref. 27 and G. Telling and S.B.P., unpublished work. Incubation times are given ±SEM.