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Title: Behavioural characterisation of a transgenic mouse model of Huntington's disease
Author: Carter, R. J.
Awarding Body: University of Cambridge
Current Institution: University of Cambridge
Date of Award: 2000
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In this thesis the behavioural phenotype of R6/2 mice was characterised thoroughly using a battery of motor and cognitive tests, so that the usefulness of these mice in testing potential therapeutic approaches to HD could be assessed. Additionally, the effect of environmental factors (such as diet and environmental stimulation) on the health and survival of R6/2 mice was investigated. Once the battery of behavioural tests was developed, and the behaviour of the mouse fully characterised, the effect of riluzole, a candidate drug for the treatment of HD and other neurodegenerative disorders, was assessed in these mice. The battery of behavioural tests revealed that R6/2 mice develop progressive cognitive and motor impairments much earlier than the overt symptoms (from 4 and 5-6 weeks of age respectively), and that the nature of these impairments is comparable to those seen in HD patients. These findings support the use of R6/2 mice as a model of HD, and indicate that both the mice and the battery of tests will be useful for the evaluation of therapeutic strategies in HD. The experiments investigating the effect of environmental factors on R6/2 mice showed that changes in the feeding regime, as well as increased environmental stimulation, significantly improved the health and survival of the mice. Finally, riluzole was shown to have a small but significant beneficial effect on the motor performance, and, under certain conditions, body weight and survival, of R6/2 mice. These results indicate that riluzole may improve the life-span and provide some symptomatic motor relief to patients with HD.
Supervisor: Not available Sponsor: Not available
Qualification Name: Thesis (Ph.D.) Qualification Level: Doctoral
EThOS ID:  DOI: Not available