Table 4.

Common animal models of Huntington’s disease.

Animal model	Name	CAG repeat length	Strengths	Weaknesses	Ref.
Mouse	R6/2	144	Rapid, progressive behavioral deficits	Limited neuropathology, short lifespan	[157]
	N171-Q82	82	Accumulation of mutant Htt aggregates	Subtle motor changes	[158]
	YAC128	128	Striatal atrophy	Late onset, subtle and transient behavior deficits	[159]
	BACHD	97	Striatal atrophy and behavioral deficits	Weight gain, late onset	[160]
	Hdh (CAG)150	150	Striatal atrophy and behavioral deficits	Late onset	[161]
Rat	TgHD51	51	Progressive behavior deficits	Late-onset and limited neuropathology	[155]
	BACHD	97	Striatal atrophy and behavioral deficits	Limited availability and late onset	[156]
	Quinolinic acid	N/A	Reproducible behavioral deficits and striatal cell loss	Not progressive, does not have the mutant Htt gene or produce mutant protein	[162]
	3-nitropropionic acid	N/A	Reproducible behavioral deficits and striatal cell loss	Does not have the mutant Htt gene or produce mutant protein	[163]
Mini-pig	N208	105	HD-like apoptotic neurons and DNA fragmentation	Limited behavioral tests and availability	[164]
Sheep	OVT73	73	Reduction in striatal GABA A receptor	Limited behavioral tests and availability	[165]
Nonhuman primate	Exon 1 HTT	84	Dystonia, chorea, neuronal inclusions and neuropil aggregates	Extremely limited availability	[166]

HD: Huntingdon’s disease.