Circadian Abnormalities in Motor Activity in a BAC Transgenic Mouse Model of HuntingtonTMs Disease ΠPLOS Currents Huntington Disease
نویسندگان
چکیده
Huntington’s disease (HD) is a progressive neurodegenerative disease marked by psychiatric and motor problems. Recently, these findings have been extended to deficits in sleep and circadian function that can be observed in HD patients and in HD mouse models, with abnormal sleep patterns correlating with symptom severity in patients. Here, we studied the behavior of the BAC HD mouse model using an 24/7 automated system; the results indicate significant lengthening of the circadian period in the mutant mice. These results reinforce previous findings in HD models and symptomatic HD patients, indicating that circadian dysfunction is a core feature of HD.
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Increased Body Weight of the BAC HD Transgenic Mouse Model of HuntingtonTMs Disease Accounts for Some but Not All of the Observed HD-like Motor Deficits ΠPLOS Currents Huntington Disease
The genome of the Bacterial Artificial Chromosome (BAC) transgenic mouse model of Huntington’s Disease (BAC HD) contains the 170 kb human HTT locus modified by the addition of exon 1 with 97 mixed CAA-CAG repeats. BAC HD mice present robust behavioral deficits in both the open field and the accelerating rotarod tests, two standard behavioral assays of motor function. BAC HD mice, however, also ...
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