Table 5.
Characteristics of representative polyQ mouse models
| Model name | Motor | Neuropathology | Cognitive | Inclusions | Lifespan | AD50 | ||||
|---|---|---|---|---|---|---|---|---|---|---|
| Age | Test (phenotype) | Age | Phenotype | Age | Test (phenotype) | Age | ||||
| HD | R6/2 | 4 | Acoustic startle PPI test (acoustic startle response reduction); open field (motor and exploratory activity decrease, rearing rate decrease); rearing climbing test (climbing ability decrease); rotarod (impairment); tail suspension test (dystonic movements) | 2 | Gene expression alteration; postnatal myelination deficit | 3.5 | Morris water maze (spatial learning and memory deficit) | 1 | 15 | 8 |
| R6/1 | 4 | Open field (motor and exploratory activity increase) | 5 | Neurotransmitter receptors level alteration | 7 | Open field habituation (habituation deficit) | 9 | 32–40 | 16 | |
| N171-82Q | 8 | Open field (rearing rate decrease) | 6 | Brain volume decrease | 13 | Rotarod (simple motor learning deficit) | 4 | 28 | 13 | |
| BACHD | 4 | Open field (rearing rate decrease); rearing climbing test (climbing ability decrease); rotarod (impairment) | 2 | Demyelination/thin myelin; postnatal myelination deficit | 12 | Light/dark choice test (anxiety increase) | 52 | Normal | 17 | |
| YAC128 | 9 | Open field (motor and exploratory activity increase) | 5 | Electrophysiology alteration | 9 | Rotarod (simple motor learning deficit) | 65 | Slightly decreased | 35 | |
| CAG140 | 4.5 | Open field (motor and exploratory activity increase, rearing rate decrease) | 8 | Brain metabolites level alteration | 6.5 | Fear conditioning test, light/dark choice test (anxiety increase) | 9 | Normal | 30 | |
| Hdh(CAG)150 | 20 | Rotarod (improvement); tail suspension test (clasping) | 26 | Brain volume decrease; cell loss; gene expression alteration; prepulse inhibition deficit | 17 | Morris water maze (spatial learning and memory deficit) | 37 | Normal | 43 | |
| SCA1 | B05 | 5 | Bar cross apparatus, open field (motor and exploratory activity increase); rotarod (impairment) | 4 | Abnormal cell morphology | 5 | Rotarod (simple motor learning deficit) | 6 | Normal | 10 |
| SCA1 154Q | 5 | Rotarod (impairment) | 16 | Brain weight loss | 5 | Rotarod (simple motor learning deficit) | 6 | 45 | 19.5 | |
| SCA3 | Q71-B | 8 | Grip strength meter (grip strength decrease); rotarod (impairment) | – | – | – | – | 13 | 36 | 17 |
| MJD84.2 | 4 | Observation (abnormal gait) | 6 | Electrophysiology alteration | – | – | +a | Normal | 41 | |
| SCA7 | PrP-SCA7-92Q | 8 | Rotarod (impairment) | 7 | Photoreceptors dysfunction | – | – | 4 | 34 | 13 |
| SCA7 266Q | 5 | Observation (muscle weakness, tremor); rotarod (impairment) | 4 | Gene expression alteration | – | – | 5 | 19 | 8 | |
| SCA17 | L7-hTBP | 9 | Observation (ataxia), rotarod (impairment) | 4 | Cell loss; cell size decrease; dendritic degeneration; loss of calbindin | – | – | – | Normal | 13 |
| DRPLA | Q129 | 3 | Observation (ataxia, seizures) | 4 | Brain volume decrease; brain weight loss; electrophysiology alteration; gene expression alteration | – | – | 9 | 16 | 8 |
| SBMA | 112Q | 6 | Tail suspension test (clasping) | 61 | Cell loss | 26 | Elevated plus/zero maze (anxiety decrease) | 6 | Normal | 30 |
| AR113Q | 8 | Grip strength meter (grip strength decrease) | 4 | Gene expression alteration | – | – | 104 | 20/110a | 13 | |
aFor further details, see “The data table”