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Huntington's disease

Huntington's disease (HD) is a hereditary neurological disorder. The average age of HD onset is about 40 years, with an average duration of illness of about 20 years. The signs of illness develops gradually and consists of changes in movement and dexterity, alterations in personality and mood, and occasionally disordered thought and impaired judgement. Eventually people with HD can no longer balance easily or to take care of themselves because of the progressive worsening of their motor functions.

HD has been definitively tied to a mutant gene and about 5-10 people in every 100 00 individuals have the clinical features of the disease. Huntington's disease develops only in individuals who have inherited the gene responsible from their mother or father. How the HD gene is triggered to cause selective brain neurons to die prematurely remains unclear. The gene is present in all tissues but only certain regions of the brain are vulnerable.

We do not have medications to halt or slow the progression of HD. There are pharmacological treatments to help suppress involuntary movements. The benefits of these drugs are often transient and they can have adverse effects such as excessively slowed movements, apathy, and problems with swallowing and speaking.

Our understanding of the HD gene has increased significantly over the last two decades, and that advance gives us hope for developing a treatment to postpone or prevent the onset of HD.