Table 3. Vulnerability to QUIN-induced neurotoxicity in HD mouse models.
Line | CAG | Dose | Age (months) |
Result vs. wt | Phenotype | Strain | Reference | |
---|---|---|---|---|---|---|---|---|
Truncated HD models | R6/1 | ~115 | 20nM | 0.75 | enhanced | presymptomatic | CBA/C57BL6 | Hansson et al., 2001 |
20nM | 2 | partial resistant | presymptomatic | Hansson et al., 2001 | ||||
20nM | 4.5 | resistant | symptomatic | Hansson et al., 2001 | ||||
20nM | 5.5/9 | resistant | symptomatic | Hansson et al., 1999 | ||||
R6/2 | ~150/200 | 20nM | 0.75 | partial resistance | presymptomatic | CBA/C57BL6 | Hansson et al., 2001 | |
20nM | 1.5 | resistant | presymptomatic | Hansson et al., 2001 | ||||
20nM | 2.5/3 | resistant | symptomatic | Mac Gibbon et al., 2002 | ||||
KA/20mg/kg | 0.75 and 2 | resistant | pre/symptomatic | Morton et al., 2000 | ||||
tg46/tg100 | 46/100 | 30nM | 4–78 | similar | presymptomatic/symptomatic | S5L/C57BL6 | Petersen et al., 2002 | |
N171 | 82 | 30nM | 4 | resistant | symptomatic | B6C3F1/J | Jarabek et al., 2004 | |
Shortstop | 120 | 4nM | 3,6,10 | resistant | no phenotype | FVB | Slow et al., 2005; unpublished | |
Full length HD modes | YAC72 | 72 | 8nM | 6,10 | enhanced | presymptomatic | FVB | Zeron et al., 2002 |
YAC128/HD55+/+ | 120 | 4nM | 3 | enhanced | presymptomatic | Graham et al., 2006 | ||
6nM | 12 | trend resistant | symptomatic | |||||
YAC128/HD53 | 120 | 4nM | 1.5 | enhanced | presymptomatic | FVB | ||
4nM | 3 | similar (trend↑) | symptomatic | |||||
6nM | 6 | similar (trend↑) | symptomatic | |||||
6nM | 10 | resistant | symptomatic |