Table 6  Commonly used SOD1-transgenic mouse models of ALS and their phenotypes in relation to transgenic expression.
SOD1 mouse models  Transgene copies  SOD1 protein levels in the CNS (human/mouse)  Disease onset (days)  Survival (months)  References
B6SJL-TgN(SOD1-G93A)1Gur  34  17  90  3.5–4.5  Gurney et al., 1994; Alexander et al., 2004
SOD1-G93A Drop Copy#3  13  –  –  6  Alexander et al., 2004
SOD1-G93A Drop Copy#4  11  –  –  6.5  Alexander et al., 2004
B6SJL-TgN(SOD1-G93A)dl1Gur  10  8–10  168  8.3  Gurney, 1997
SOD1-G93A Drop Copy#1  4  –  –  21  Alexander et al., 2004
G37R  –  4–12  105  5  Wong et al., 1995; Haenggeli et al., 2007
G85R  –  0.2–1  240  8.5  Bruijn et al., 1997
G86R (M1 line)  –  –  90–120  4  Ripps et al., 1995
D90A  –  –  350  13.5  Jonsson et al., 2006
(–), unknown.