Table 2.
Genotype | Cell Culture Model | Sex | Age at Biopsy (Years) | Mutation | Family History | Age of Disease Onset | Clinical Phenotype | Disease Duration (Months) |
---|---|---|---|---|---|---|---|---|
Controls | hiPSC | |||||||
female | 48 | - | - | - | - | - | ||
male | 60 | - | - | - | - | - | ||
female | 45 | - | - | - | - | - | ||
female | 50 | - | - | - | - | - | ||
FUS-ALS | hiPSC | |||||||
female | 58 | p.R521C * | Pos. for ALS | 57 | spinal | 7 | ||
female | 65 | p.R521L * | Pos. for ALS | 61 | spinal | 60 | ||
SOD1-ALS | hiPSC | |||||||
Male | 59 | p.R115G | Pos. for ALS | n.a | spinal | n.a | ||
female | 46 | p.D90A * | Pos. for ALS | n.a | Spinal | n.a |
n.a: not available; * two clones were used in the current study.