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