Table 1.
Protein expression and survival data for PrP-SMN and HSA-SMN transgenic SMA animals
| Line | Copy no. | Protein expression | Mean lifespan in days | |||||
|---|---|---|---|---|---|---|---|---|
| Brain | Spinal cord | Heart | Muscle | Liver | Heterozygous | Homozygous | ||
| PrP-SMN | ||||||||
| 92 | 8–9 | +++ | +++ | − | + | + | 150 ± 100 (n = 21) | 210 ± 97 (n = 46) |
| 13 | 1 | − | − | − | − | − | 7.0 ± 0.7 (n = 36) | Maximum 28 |
| HSA-SMN | ||||||||
| 63 | 5 | + | + | +++ | +++ | + | 6.6 ± 0.8 (n = 29) | 160 ± 33.9 (n = 20) |
| 69 | 11 | − | − | − | +++ | − | ND | 3.5 ± 0.5 (n = 15) |
| Controls | ||||||||
| SMA | + | − | − | − | − | 4.6 ± 0.4 (n = 33) | ||
| Carrier | ++ | ++ | + | + | + | Normal (21) | ||
| High copy | +++ | +++ | ++ | ++ | ++ | Normal (21) | ||
For protein expression data PrP92, PrP13, HSA63, HSA69, animals are all homozygous for the HSA-SMN or PrP-SMN transgene, SMN2 and do not contain mouse Smn. Protein expression determined by western blot analysis on tissue from PND05 animals.
Copy no. refers to the number of copies of the PrP or HSA transgenes.
‘−’ not detectable, ‘+’ low, ‘++’ medium, ‘+++’ high expression, ND = not determined, n = number of mice.
For mean lifespan in days heterozygous and homozygous refer to the transgene, all animals are homozygous for SMN2 and do not contain mouse Smn.