Table 1.
Characteristics of different mouse models of Gaucher disease at 6 mo of age
| Gba+/+ | Gba1D409V/+ | Gba1D409V/D409V | Gba1+/− | |
| Glucocerebrosidase activity (ng/h/mg of protein) | 19.2 ± 1.3 (n = 8) | 11.3 ± 1.2 (n = 7)* | 3.6 ± 0.4 (n = 7)# | 10.4 ± 0.8 (n = 7)* |
| GlcCer (ng/mg wet tissue) | 5.9 ± 0.5 (n = 7) | 6.2 ± 0.8 (n = 7) | 6.3 ± 0.8 (n = 7) | 6.1 ± 0.6 (n = 7) |
| GlcSph (ng/mg wet tissue) | 0.32 ± 0.06 (n = 7) | 0.29 ± 0.08 (n = 7) | 0.73 ± 0.21 (n = 7)* | 0.29 ± 0.09 (n = 7) |
| α-Synuclein aggregates† | 0.04 ± 0.01% (n = 8) | 0.15 ± 0.03% (n = 5)* | 0.23 ± 0.04% (n = 7) # | 0.05 ± 0.02% (n = 5) |
| Hippocampal memory‡ | 71 ± 2% (n = 7)§ | 72 ± 4% (n = 6)§ | 47 ± 5% (n = 7) | 71 ± 3% (n = 7)§ |
*,#Groups with different symbols are significantly different from each other using one-way ANOVA followed by Newman-Keuls (P < 0.05). All data are expressed as mean ± SEM.
†Proteinase K-resistant α-syn aggregates are represented as percent-threshold area in the hippocampus.
‡Data for memory function represents the percentage of target investigations on the testing day (T2).
§Wild-type (Gba1+/+), Gba1D409V/+, and Gba1+/− mice showed a significant preference for the novel object (target investigations > 50%) on the testing day using one-sample t test (P < 0.01).