Table 1. First passage VPSPr inoculations to Tg(HuPrP) harboring the same PrP 129 genotype as the inoculum*.
| 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 1st‡ | (129V)×8§ |
0/11 | 3/9 | 741 ± 76/741 ± 55 | 4/11 | 768 ± 74/733 ± 71 | |
| VPSPr-129VV 2nd | 0/17 | 10/12 | 711 ± 65/722 ± 59 | 6/17 | 635 ± 204/682 ± 131 | ||
| VPSPr-129VV 3rd |
0/11 |
3/8 |
669 ± 92/651 ± 89 |
|
3/10 |
642 ± 157/604 ± 140 |
|
| VPSPr-129VV 4th | (129V)×3 |
0/9 | 7/9 | 655 ± 67/642 ± 104 | NA | NA | |
| VPSPr-129VV 5th |
0/6 |
3/6 |
541 ± 96/642 ± 76 |
|
NA |
NA |
|
| VPSPr-129MM 1st | (129M)×2 | 0/6 | 4/6 | 678 ± 90/590 ± 154 | NA | NA | |
| VPSPr-129MM 2nd (presymptomatic) | 0/6 | 0/6 | 0/746 ± 59 | NA | NA | ||
Dpi, days postinoculation; M, methionine; V, valine; NA, not available; VPSPr, variably protease-sensitive prionopathy associated with homozygous 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.