Table A1.

List of Huntington’s disease models used in the reviewed studies.

Model	Description	Reference
CAG140 KI	knock-in murine model expressing full-length Hdh gene with exon1 from human HTT with 140 CAG repeats	[79]
HD-hiPSCs	human-induced pluripotent stem cells derived from HD patients differentiated towards medium spiny neurons	[32]
Htt-72Q	murine primary cortical neuron model expressing HTT exon1 with 72 CAG repeats	[8]
OVT73	transgenic sheep model expressing full-length human HTT cDNA with 73 CAG/CAA repeats under the control of the human promoter	[80]
R6/1	murine model transgenic for the 5′ end of the HTT gene carrying 116 CAG repeats	[81]
R6/2	murine model transgenic for the 5′ end of the HTT gene carrying 144 CAG repeats	[81]
N171-82Q	Transgenic murine model expressing N-terminal truncated human HTT cDNA with 82 CAG repeats under the influence of mouse prion protein promoter	[82]
STHdhQ111	cell culture model derived from knock-in mouse embryos expressing full-length Hdh 1 with 111 CAG repeats	[83]
YAC128	yeast artificial chromosome transgenic murine model expressing full-length HTT transgene with 128 CAG repeats	[84]
zQ175	knock-in murine model expressing full-length Hdh gene with exon1 from human HTT with 188 CAG repeats, derived from CAG140 KI	[85]

¹ Murine homolog of human HTT.