Table 1.

Other transgenic mouse models of HD

Model name	Promoter	CAG number	Properties	References
D9-N171-98Q	mDarpp-32	98	Cell-autonomous MSNs dysfunction, mild phenotype	[23]
HD48, HD89	CMV	48, 89	Full HTT cDNA; relatively slow, biphasic phenotype; decreased lifespan; neuronal loss, clasping and circling behavior	[25, 300]
HD 100	rat Eno2	100	Neuron-specific model; corticostriatal pathway impairment	[273, 313]
HD150QG, HD 190QG	HTT (1 kb of 5′UTR)	150, 190	EGFP-HTT model; R6-like phenotype; decreased life span; possible attenuating effect of eGFP on disease severity	[311]
NLS144, NES144	HTT (1 kb of 5′UTR)	143	Modified R6/2 constructs with either NLS or NES signals produce accelerated and ameliorated phenotypes, respectively	[276]
Htt-160Q	GFAP (Gfa2)	160	Glia-specific expression of N-terminal HTT; decreased lifespan; non-cell-autonomous mechanism of HD pathogenesis	[231]
NLS-N171-82Q	PrP	82	NLS signal ameliorates the phenotype of N171 mice	[22]
N118-82Q	PrP	82	Shorter N-terminal HTT fragment; N171-like phenotype with earlier premature death	[24]
N586-82Q/K14-eGFP	PrP	82	N-terminal HTT (equivalent to caspase-6 clevage product); phenotype milder than N171 with unexpected cerebellar pathology, dyskinesia and ataxia; frequent cytoplasmic aggregates	[236]
N586-82Q	PrP	82	N-terminal HTT (equivalent to caspase-6 clevage product)	[237]
shortstop	HTT	120	N-terminal YAC model; no disease phenotype; NIIs present	[20]
BACHD-SD, BACHD-SA	HTT	97a	Mutation of HTT at serines 13 and 16 to either phosphomimetic (SD) or phosphoresistant (SA) version; mice with normal and BACHD-like phenotype, respectively	[239]
HD94	CamKIIα	94	Forebrain-specific conditional (dox-regulated) model; reversal of NIIs and HD-like phenotype upon HTT expression turn off	[35]
Prp-tTA-6/iFL148Q-69	PrP	148	Conditional model; full-length HTT; phenotype similar to other N-terminal models, but slower disease progression rate; decreased lifespan	[37]
RosaHD/Dlx5/6-Cre	Rosa26/Dlx5/6	103	Restriction of HTT expression to MSNs; changes in NMDA receptor-mediated currents; no other neurological phenotypes	[233]
RosaHD/Emx1-Cre	Rosa26/Emx1	103	Restriction of HTT expression to cortical pyramidal neurons; no neurological phenotype; polyQ nuclear accumulation	[36]
RosaHD/Nestin-Cre	Rosa26/Nestin	103	Restriction of HTT expression to neurons; evident neuropathology; locomotor activity decrease	[36]

^aMixed CAA/CAG repeats