Table 2.
Mean and standard deviation (SD) of number of sense and antisense RNA foci per nucleus in Purkinje neurons, granule neurons, motor neurons, dentate gyrus neurons, and CA4 subfield neurons in four C9ORF72-ALS patients
| Case | Antisense (mean) | Antisense (SD) | Sense (mean) | Sense (SD) | p value |
|---|---|---|---|---|---|
| Purkinje neurons | |||||
| 1 | 26.40 | 20.3 | 6.40 | 17.4 | 2.37E−14 |
| 2 | 4.30 | 4.19 | 1.10 | 2.18 | 0.002 |
| 3 | 4.60 | 4.50 | 1.30 | 1.95 | 0.002 |
| 4 | 6.30 | 5.54 | 1.40 | 1.51 | 6.88E−05 |
| Granule neurons | |||||
| 1 | 0.00 | 0.00 | 0.56 | 1.20 | 1.37E−12 |
| 2 | 0.03 | 0.17 | 1.10 | 1.41 | 9.63E−18 |
| 3 | 0.01 | 0.10 | 0.34 | 0.93 | 1.17E−07 |
| 4 | 0.02 | 0.14 | 0.40 | 0.82 | 3.1E−07 |
| Motor neurons | |||||
| 1 | 14.90 | 24.5 | 1.50 | 1.65 | 5.65E−14 |
| 2 | 3.00 | 4.06 | 1.00 | 1.25 | 0.02 |
| 3 | 3.33 | 3.78 | 1.00 | 0.76 | 0.02 |
| 4 | 5.40 | 6.52 | 2.44 | 3.40 | 0.02 |
| Case | Antisense (mean) | Antisense (SD) | Sense (mean) | Sense (SD) |
|---|---|---|---|---|
| Dentate gyrus neurons | ||||
| 1 | 0.65 | 1.90 | 0.88 | 2.34 |
| 6 | 1.65 | 3.35 | 0.95 | 1.81 |
| 7 | 0.89 | 2.42 | 1.39 | 2.23 |
| 8 | 1.63 | 5.34 | 0.91 | 1.76 |
| CA4 subfield neurons | ||||
| 1 | 10.3 | 14.7 | 6.55 | 8.68 |
| 6 | 3.17 | 6.51 | 1.33 | 1.67 |
| 7 | 0.50 | 0.97 | 2.72 | 4.90 |
| 8 | 6.31 | 10.8 | 9.82 | 13.8 |
In each case, antisense RNA foci are significantly more numerous in Purkinje neurons and motor neurons (likelihood-ratio test p < 0.05) but significantly less numerous in granule neurons (likelihood-ratio test p < 0.05)