Table 4.

Common animal models of Huntington’s disease.

Model	Characteristics	Reference
1. Mouse models
Genetically modified models
R6/2	Expresses human exon 1 of HTT with ~150 glutamine repeats	Mangiarini et al. (1996)
R6/1	Expresses human exon 1 of HTT with ~115 glutamine repeats	Mangiarini et al. (1996) and Naver et al. (2003)
N171-82Q	Expresses a 171 amino acid mutant HTT fragment with 82 glutamine repeats	Schilling et al. (1999)
YAC128	Expresses full length human HTT with 128 glutamine repeats	Slow et al. (2003) and Van Raamsdonk et al. (2005)
BACHD	Expresess full lenght human HTT with 97 glutamine repeats	Gray et al. (2008)
HdhQ111	knock-in mouse having human HTT exon 1 sequence with 111 glutamine repeats	Wheeler et al. (2000)
HdhQ140	knock-in mouse having human HTT exon 1 sequence with 140 glutamine repeats	Menalled et al. (2003)
HdhQ150	knock-in mouse having mouse HTT exon 1 sequence with 150 glutamine repeats	Lin et al. (2001)
Pharmacological models
Quinolonic acid (QA)	QA directly administered to the striatum induces striatal neurodegeneration	McLin et al. (2006)
3-Nitropropionic acid (3-NP)	Repeated injections of 3-NP produce excitotoxic-like lesions of the striatum	Gould et al. (1985)
2. Rat models
Pharmacological models
Quinolonic acid (QA)	QA directly administered to the striatum induces striatal neurodegeneration	Bordelon et al. (1997)
3-Nitropropionic acid (3-NP)	Repeated injections of 3-NP produce excitotoxic-like lesions of the striatum	Gould et al. (1985)
Kainic acid	Intrastriatal injection produces selective degeneration of neurons	Coyle et al. (1978)
Ibotenic acid (IBO)	Central microinjections induce lesions in the striatum	Smith et al. (1987)
3. Larger animal models
Pharmacological models
HD rhesus monkey	Expresses mutant exon 1 HTT with 84 glutamine repeats	Yang et al. (2008)
HD pigs (N208-105Q)	Expresses 208 N-terminal aminoacids of mutant HTT with 105 glutamine repeats	Yang et al. (2010)