Mouse Models of Huntington's Disease

In this review, we explore the similarities and differences in the behavioural neurobiology found in the mouse models of Huntington's disease (HD) and the human disease state. The review is organised with a comparative focus on the functional domains of motor control, cognition and behavioural disturbance (akin to psychiatric disturbance in people) and how our knowledge of the underlying physiological changes that are manifest in the HD mouse lines correspond to those seen in the HD clinical population. The review is framed in terms of functional circuitry and neurotransmitter systems and how abnormalities in these systems impact on the behavioural readouts across the mouse lines and how these may correspond to the deficits observed in people. In addition, interpretational issues associated with the data from animal studies are discussed.