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.