Review Article
Recent Trends in Detection of Huntingtin and Preclinical Models of Huntington’s Disease
Table 2
Preclinical models of Huntington’s disease (reproduced with permission from van Raamsdonk et al. [
18]).
| | Human HD symptoms | Cell models | Worm models | Fly models | Neurotoxin models (mouse/rat/monkey) | N-Terminal models (mouse/rat) | Knock in mouse models | Full-length mouse models |
| | Motor deficits | n/a | Yes | Yes | Yes | Yes | Yes | Yes | | Cognitive impairment | n/a | n/a | n/a | Yes | Yes | No | Yes | | Atrophy | Yes | n/a | n/a | Yes (selective) | Yes | Yes (selective) | Yes (selective) | | Neuronal loss | Yes | No | Yes | Yes (selective) | Yes/no | No | Yes (selective) | | Progressive phenotype | n/a | No | Yes | No | Yes | Yes | Yes | | Full-length mutant Htt | Yes/no | No | Yes/no | No | No | Yes | Yes | | Studies of pathogenesis | Yes | Yes | Yes | No | Yes | Yes | Yes | | Screening assays for therapeutic compounds | Yes | Yes | Yes | Yes | No | No | No | | Therapeutic trials | No | No | No | Yes | Yes | No | Yes |
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