Table 3.
iPSC-derived in vitro studies to model ALS and analyse disease-related mechanisms and phenotypes.
| ALS patient-derived iPSCs: genetic mutation | differentiation into cell type | differentiation marker | result | references |
|---|---|---|---|---|
| C9orf72 | motor neurons | Tuj-I | C9orf72 DPR aggregation | [75] |
| C9orf72 RNA Foci | ||||
| glutamate excitotoxicity | ||||
| hyperexcitability | ||||
| TDP-43 | motor neurons | Isl- I | TDP-43 aggregation | [76] |
| Tuj-I | cytoplasmic granules | |||
| short neurites | ||||
| TDP-43 | motor neurons | HB9 | immature neurite growth | [77] |
| SOD-1 | ChAT | action potential irregularities | ||
| C9ORF72 | SMI-32 | SOD1 aggregation | ||
| autophagy dysfunction |