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