Table 1. DpI mRNA expression levels and onset of clinical phenotype.
| Gene copy number
|
Expression level*
|
Age at onset of ataxia†
|
||||
|---|---|---|---|---|---|---|
| Mouse line | Prnp+/+ ZH I | Prnp+/0 ZH I | Prnp0/0 ZH I | Prnp+/0 ZH I/Tg20 | ||
| Tg15 | 10x | 100 | 4-8 (n = 10) | n/a | n/a | 4-8 (n = 10) |
| Tg16 | 3x | 82 | 4-8 (n = 10) | n/a | n/a | 4-8 (n = 10) |
| Tg79 | 2x | 14 | >97 (n = 7) | >80-90 (n = 10) | ≈22 (n = 8) | n/a |
| Tg80 | 1x | 10 | >97 (n = 3) | >68-90 (n = 7) | >57-75 (n = 8) | n/a |
n/a, not applicable.
*
DpI mRNA expression level, expressed in percent of levels of tg 15 mice, determined by Northern blot analysis of the cerebellum of all mouse lines. Transgene copy number and development of the phenotype were assessed as described in Materials and Methods.
†
Interval of age in weeks when 100% of the mice analyzed showed the phenotype.