Table 2. First passage VPSPr inoculations to Tg(HuPrP) mice harboring different PrP 129 genotypes from those of the inocula*.
| Inoculum | Tg(HuPrP) | No. with clinical signs/total | Histology, immunohistochemistry |
PrPSc Western blot |
|||
|---|---|---|---|---|---|---|---|
| No. positive/total | Dpi positive/total† | No. positive/total | Dpi positive/total | ||||
| VPSPr-129VV 6th‡ | (129M)×1§ |
0/5 | 0/5 | 0/643 ± 71 | NA | NA | |
| VPSPr-129VV 4th | 0/4 | 0/4 | 0/659 ± 55 | NA | NA | ||
| VPSPr-129VV 5th |
0/5 |
0/5 |
0/757 ± 57 |
|
NA |
NA |
|
| VPSPr-129MV 1st | (129M)×2 |
0/9 | 0/9 | 0/745 ± 70 | NA | NA | |
| VPSPr-129MV 2nd | 0/6 | 0/6 | 0/582 ± 101 | NA | NA | ||
| VPSPr-129MV 3rd |
0/4 |
0/4 |
0/694 ± 153 |
|
NA |
NA |
|
| VPSPr-129MV 4st | (129V)×8 | 0/6 | 0/6 | 0/586 ± 68 | NA | NA | |
| VPSPr-129MV 3rd | 0/7 | 0/7 | 0/681 ± 102 | NA | NA | ||
Dpi, days postinoculation; M, methionine; V, valine; NA, not available; VPSPr, variably protease-sensitive prionopathy associated with homozygosity V or M at codon 129 of the PrP gene (VPSPr-129VV or VPSPr-129MM). †Dpi of positive mice compared with dpi of all mice (including positive mice). Each value represents the mean ± SD. ‡Identifies the VPSPr case-patients providing the inoculum, i.e., inocula were obtained from 5 case-patients with VPSPr-129VV and 2 with VPSPr-129MM. §Refers to the presence of V or M at PrP residue 129 and expression level indicated as × normal.