Table 2.
Enzymatic Activity and Protein Detection Within the NHP Thalamus
| NHP ID (dose, survival) | Enzymatic activity (nmol/hr/mg protein) | Percentage of enzymatic activity above endogenous levels (fold) |
|---|---|---|
| Controls (3m)a | 47.7 | n/a |
| NHP 3 (LD, 3m) | 64.6 | 35 (1.4-fold) |
| NHP 5 (HD, 3m) | 144 | 202 (3.0-fold) |
| NHP 6 (HD, 3m) | 351 | 636 (7.4-fold) |
| NHP 7 (LD, 9m) | 340 | 612 (7.1-fold) |
| NHP 8 (HD, 9m) | 418 | 776 (8.8-fold) |
3m, 3 months; 9m, 9 months; L/HD, low/high dose; +/−, band present/absent in Western blot; n/a, not applicable.
a
Enzymatic activity for controls (3m) is represented by the mean from two PBS-control animals (NHPs 1 and 2). Note that NHP 4 was not included in this analysis as the 6-mm punch was taken outside the area of ASM expression, and thus no enzyme activity was detected (see Supplementary Fig. S2).