Table 2.
Regional distribution of Lewy-body-like protein aggregates in brains of LSD model mice.
| α-Synuclein | Tau | MAP2(2a + 2b) | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| HC | CB | CX | BS | HC | CB | CX | BS | HC | CB | CX | BS | |
| Gaucher * | 100% | 100% | 100% | 0 | 100% | 100% | 100% | 0 | 100% | 100% | 100% | 0 |
| Fabry | 40% | 40% | 0 | 0 | 100% | 100% | 0 | 0 | 40% | 40% | 0 | 0 |
| Sandhoff | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| NPA | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| Hurler | 20% | 20% | 0 | 0 | 100% | 100% | 0 | 60% | 20% | 20% | 0 | 0 |
| Pompe | 0 | 0 | 0 | 0 | 60% | 80% | 40% | 100% | 0 | 0 | 0 | 0 |
| NPC | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
Percent of mice exhibiting protein aggregates are shown. n = 5 per model. * Data from Gaucher model from historical data (Sardi et al., 2011; Sardi et al., 2013). See Methods for experimental details regarding staining and blinded semiquantitative assessment. Abbreviations: HC = hippocampus, CB = cerebellum, CX = cortex, BS = brainstem.