Analysis Tools
behavior/neurological
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• 2 month old mice exhibit increased falls and decreased latency to fall during rotarod training, indicating a motor learning deficit
• males show an overall greater impairment on the rotarod than males homozygous for Tg(YAC18)18Hay and Htttm1Hay
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• at 9 months of age, mice show deficits in object recognition by preference for an unknown object
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• at 6 and 9 months of age, mice show a spatial deficit in spatial learning by preference for a known object in a novel location
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• mice spend more time being immobile and less time swimming in the Porsolt forced swim test compared to mice homozygous for Tg(YAC18)18Hay and Htttm1Hay, indicating increased depressive-like behavior
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• during open-field exploration, mice enter the center of the field less frequently and spend less total time in the center of the field compared to mice homozygous for Tg(YAC18)18Hay and Htttm1Hay, indicating increased anxiety
• in the elevated plus maze, mice spend less time in the open arms and dip their head off the edges of the open arms less frequently than mice homozygous for Tg(YAC18)18Hay and Htttm1Hay, indicating increased anxiety
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• during a spontaneous climbing test, 2 month old mice trend toward an increased latency to begin climbing and decreased number of climbing events and spend significantly less time climbing than mice homozygous for Tg(YAC18)18Hay and Htttm1Hay
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• in longitudinal rotarod testing, mice exhibit declining performance with age
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• mice show increased stereotypy, or repetitive movement, and decreased jumping compared to mice homozygous for Tg(YAC18)18Hay and Htttm1Hay
• however, no difference in distance traveled or ambulation time is seen during spontaneous activity
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growth/size/body
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• increase in body weight compared to wild-type controls and a small increase in body weight compared to mice homozygous for Tg(YAC18)18Hay and Htttm1Hay
• body weight gain is greater in females than in males
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nervous system
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• mice show a trend towards reduced forebrain weight at 12 months of age
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• mice show a trend toward reduced corpus callosum volume at 12 months of age
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• mice show reductions in both striatal volume and cortical volume at 12 months of age
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• at 9 months of age, striatal spiny projection neurons show a significant depolarization of the resting membrane potential and elevated action potential threshold, however excitability of these neurons is similar to controls
• action potential threshold of striatal spiny projection neurons is already increased at 6 months of age
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• mice exhibit progressive hippocampal synaptic plasticity deficit
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• at 9 months of age, striatal spiny projection neurons exhibit small changes in membrane properties
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• at 9 months of age, striatal spiny projection neurons exhibit lower amplitude and frequency of spontaneous excitatory postsynaptic currents (sEPSCs)
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• at 9 months of age, striatal spiny projection neurons exhibit lower frequency of spontaneous excitatory postsynaptic currents (sEPSCs)
• however, release probability from presynaptic terminals is unaltered
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• mice exhibit a deficiency in long-term potentiation at CA3-to-CA1 synapses at 9 months of age but not at 3 months of age
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• paired-pulse facilitation in the hippocampus is reduced at 9 months of age indicating impaired short-term plasticity
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Huntington's disease | DOID:12858 |
OMIM:143100 |
J:191147 , J:215223 | |
