Table 2 Summary of the demographics of the four subtypes in AALS cohort
ProS1 | ProS2 | ProS3 | ProS4 | p value | |
|---|---|---|---|---|---|
Donors (number) | 21 | 40 | 48 | 60 | - |
Age at Symptom Onset(years) | 62(31–75) | 56(25–79) | 52(25–73) | 54(35–73) | 0.0064 |
unknown | 4.70% | - | 4.20% | 1.70% | - |
Age at Sample Collection | 64(33–77) | 59(49–79) | 56(29–76) | 58(43–82) | 0.0382 |
unknown | 2.5% | 22.9% | 20% | ||
iPSC cell origin | |||||
T cell | 52.3% | 62.5% | 47.9% | 55% | 0.8321 |
Non-T cell | 38% | 37.5% | 52.1% | 45% | 0.7385 |
Disease duration (years) | 3.1(1–6) | 3.2(1–9) | 4.4(2–20) | 5.5(1–16) | 0.2462 |
unknown | 42.80% | 50% | 58.30% | 56.60% | - |
Sex female | 38% | 90% | 29.10% | 23.30% | 2.91e-06 |
Site of Onset | |||||
Limb | 52.30% | 65% | 56.20% | 75% | 0.5699 |
Bulbar | 33.30% | 27.30% | 20.80% | 11.60% | 0.1811 |
Multiple | 14.50% | 7.50% | 18.70% | 10% | 0.4456 |
Axial | - | - | - | 1.60% | - |
Other | - | - | 4.10% | 1.60% | - |
ALSFRS-R Progression Slope | −0.69(−0.56 ~ 0.33) | −0.71(−1.88 ~ 0.03) | −0.69(−2.93 ~ 0.11) | −0.46(−1.75 ~ 0.25) | 0.3109 |
unknown | 42.8% | 42.5% | 27.0% | 38.3% | - |
CBS Progression Slope | 0.118(−0.53 ~ 0.66) | −0.04(−1.5 ~ 0.875) | 0.07 (−0.34 ~ 0.58) | −0.02(−0.48 ~ 0.34) | 0.5281 |
unknown | 61.9% | 60.0% | 60.4% | 70.0% | - |
Self reported mutations | |||||
C9orf72 | 14.3% | 17.5% | 8.3% | 3.3% | 0.1277 |
TARDBP | - | - | 2.0% | - | - |
SOD1 | 4.7% | - | 2.0% | 8.3% | 0.3791 |
others | 4.7% | - | 2.0% | 3.3% | 0.8363 |
Unknown | 76.3% | 82.5% | 85.7% | 85.1% | - |
C9orf72 repeats | |||||
normal repeats(≤25) | 19.1% | 22.5% | 12.6% | 16.7% | 0.7234 |
high repeats(>25) | 4.8% | 17.5% | 2.0% | 0.0% | 0.002 |
NA | 76.1% | 60.0% | 85.4% | 83.3% | - |
