Table 1.
Animal models for HD mentioned in the review.
| Name | Type | DNA Construct | CAG Repeats Length | AGE of Onset | Mutant HTT Expression* | Reference |
|---|---|---|---|---|---|---|
| R6/2 mouse | Transgenic | Promoter and exon 1 of human Htt gene | 144 | 9–11 weeks (juvenile HD) |
75% | [43] |
| R6/1 mouse | Transgenic | Promoter and exon 1 of human Htt gene | 116 | 4–5 months | 30% | [43] |
| NLS-N171-82Q mouse | Transgenic | Mouse prion promoter and human sequence for the first 171 N-terminal amino acids of Htt | 82 | 9–11 weeks (juvenile HD) |
10–20% | [44,45,46,47] |
| HdhQn mouse | Knock-in | Knock-in of variable number of CAG repeats into mouse Htt locus | 92–200 | Depending on the length of the CAG repeats | 100% | [40,41,42,48,49] |
| BACHD mouse |
Transgenic | Full-length human Htt gene under human Htt promoter | 97 | Slow disease progression and normal life span | 100% | [50,51] |
| YACQn mouse | Transgenic | Full-length human Htt gene with variable number of CAG repeats under human Htt promoter | 48–128 | Depending on the length of the CAG repeats | 30–100% | [52,53,54,55] |
| Mini pigs | Transgenic | Human promoter and human sequence for the first 548 N-terminal amino acids of Htt | 124 | Slow progression of the disease | 100% | [56] |
Qn: variable (n) glutamine repeats; BAC: bacterial artificial chromosome; YAC: yeast artificial chromosome vector system; Htt: Huntingtin. *relative to endogenous level.