Table 5.
SOD1, TDP-43 and FUS mouse models of ALS.
| Mouse models | Promotor | CNS over-expression (fold) | Survival (months) | Inclusions | Motor Phenotype | MN loss | Denervation | References | |
|---|---|---|---|---|---|---|---|---|---|
| SOD1 | G93A | hSOD1 | 17 | 3.5–4.5 | SOD1(+) | Yes | Yes | Yes | Gurney et al., 1994 |
| s-G93A | hSOD1 | 8–10 | 8.3 | hyaline | Yes | Yes | Yes | Gurney, 1997 | |
| G37R | hSOD1 | 4–12 | 5 | SOD1(+) | Yes | Yes | Yes | Wong et al., 1995 | |
| G85R | hSOD1 | 0.2–1 | 8.5 | SOD1(+) Ub(+) | Yes | Yes | Yes | Bruijn et al., 1997 | |
| TDP-43 | A315T | PrP | 3 | 5 | TDP-43(–) Ub(+) | Yes | Yes | Yes | Wegorzewska et al., 2009 |
| rNLS8 | NEFH | – | 2.6 off Dox | TDP-43(+) | Yes | Yes | Yes | Walker et al., 2015; Spiller et al., 2016a | |
| M337VKNOCK-IN | – | No | 24.5 | No | No | No | Yes | Ebstein et al., 2019 | |
| G298SKNOCK-IN | – | No | 24.5 | No | No | Yes | Yes | Ebstein et al., 2019 | |
| TDP-43 KO | – | – | ns | No | Yes | Yes | Yes | Iguchi et al., 2013 | |
| FUS | hFUSWT | MAPT | 2.6 | No | No | No | Yes | Sharma et al., 2016 | |
| hFUSR521C | MAPT | 4 | 12 | No | No | Yes | Yes | Sharma et al., 2016 | |
| hFUSP525L | MAPT | 4 | 12 | No | Yes | Yes | Yes | Sharma et al., 2016 | |