Table 3.
Mean and standard deviation (SD) of number of inclusions per cell containing poly-GA and poly-PA protein, in granule neurons and motor neurons from six patients with C9ORF72-disease
| Case | Poly-PA (mean) | Poly-PA (SD) | Poly-GA (mean) | Poly-GA (SD) | p value |
|---|---|---|---|---|---|
| Motor neurons | |||||
| 1 | 2.1 | 2.32 | 0 | 0 | 0 |
| 2 | 2.4 | 3.58 | 0.5 | 0.76 | 1.57E−34 |
| 4 | 2 | 1.83 | 0.3 | 0.35 | 0.0046 |
| 6 | 1.2 | 0.75 | 0 | 0 | 1.55E−21 |
| 7 | 1.7 | 1.56 | 0.2 | 0.40 | 0 |
| 8 | 4.4 | 2.07 | 0.2 | 0.44 | 0 |
| Granule neurons | |||||
| 1 | 0.04 | 0.18 | 0.2 | 0.36 | 2.42E−44 |
| 2 | 0.01 | 0.07 | 0.21 | 0.37 | 0 |
| 4 | 0.04 | 0.2 | 0.2 | 0.42 | 0 |
| 6 | 0.01 | 0.09 | 0.1 | 0.36 | 0 |
| 7 | 0.01 | 0.08 | 0.1 | 0.29 | 7.58E−49 |
| 8 | 0.01 | 0.12 | 0.1 | 0.34 | 0 |
In each case, poly-GA containing inclusions are significantly more numerous in granule neurons (likelihood-ratio test p < 0.01) and poly-PA containing inclusions are significantly more numerous in motor neurons (likelihood-ratio test p < 0.01)