Table 1.

Commonly used mouse models of Huntington’s Disease.

Strain Name	Transgenic or Knockin	Gene Characteristics	Promoter	Repeat Length	Motor Symptom Onset	Lifespan	Background Strain(s)	References
R6/2	Transgenic fragment	Exon 1 of human HTT gene	1 kb of Human HTT	~150	6 weeks	10–13 wks	C57BL/6XCBA	Mangiarini et al., 1996; Carter etal., 1999
R6/1	Transgenic fragment	Exon 1 of human HTT gene	1 kb of Human HTT	116	18 weeks	32–40 wks	C57BL/6xCBA or C57BL/6	Mangiarini et al., 1996; Hodges et al., 2008
N171-82Q	Transgenic fragment	First 171 AA of human HTT (exons 1, 2, part of 3)	Prnp	82	3 months	16–22 wks	C57BL/6xC3H/He	Schilling et al., 1999; Schilling et al., 2004
Tg100	Transgenic fragment	First ~3 kb of human HTT cDNA	Rat NSE	100	3 months (nonspecific)	Normal	C57BL/6xSJL	Laforet et al., 2001
HD94	Transgenic fragment	Chimeric human/ mouse HTT exon 1	TetO + tTA	94	4–8 weeks (clasping)	Normal	C57BIV6xCBA	Yamamoto et al., 2000
YAC72	Transgenic full-length	Full length human HTT gene	Human HTT	72	16 months	Normal	FVB/N	Hodgson et al., 1999; Seo et al., 2008
YAC128	Transgenic full-length	Full length human HTT gene	Human HTT	120	6 months	Normal	FVB/N	Slow et al., 2003
BACHD	Transgenic full-length	Full length human HTT gene (floxed exon 1)	Human HTT	97 (mixed)	2 months	Normal	FVB/N	Gray et al., 2008
HdhQ72, Q80	Knockin	Endogenous murine Htt gene, expanded CAG inserted	Mouse Htt	72,80	12 months	Normal	Mixed 129Sv, C57BIV6	Shelboume et al., 1999; Kennedy et al., 2003
HdhQ111	Knockin	Endogenous murine Htt gene, chimeric human/mouse exon 1	Mouse Htt	109	24 months (gait)	Normal	Mixed 129Sv, CD1	Wheeler et al., 1999; Wheeler et al., 2002
HdhQ94	Knockin	Endogenous murine Htt gene, chimeric human/mouse exon 1	Mouse Htt	94	2 months (rearing)	Normal	Mixed 129Sv, C57BL/6	Levine et al., 1999; Menalled et al., 2002
HdhQ140	Knockin	Endogenous murine Htt gene, chimeric human/mouse exon 1	Mouse Htt	140	4 months	Normal	Mixed 129Sv, C57B176	Menalled et al., 2003; Hickey et al., 2008
HdhQ150	Knockin	Endogenous murine Htt gene, expanded CAG inserted	Mouse Htt	150	100 weeks	Normal	Mixed 12901a, C57BIV6	Lin et al., 2001; Heng et al., 2007