The UK DRI is committed to studying all the dementias. In moving towards this goal, we are excited to announce that we have awarded a new programme in Huntington’s disease. The £1.5m programme will be led by Professors Gillian Bates and Sarah Tabrizi from University College London (UCL) and will look to better understand the DNA damage that occurs as this neurodegenerative disease unfolds as well as the potential for better targets for treatment.
Huntington’s disease is an inherited condition caused by a faulty gene in our DNA which affects the nervous system and can impact movement, learning, thinking and emotions. The disease usually progresses and gets worse over a 10-25 year period from when it initially appears. It was previously thought that 4-6 people in a population of 100,000 were affected by Huntington's disease. However, UK research carried out in 2012 found the actual figure for those affected by the condition to be about 12 people per 100,000. It's estimated that the number of people who have the Huntington's gene and are not yet affected is about twice that of those who have symptoms.
Exciting progress has been made in identifying potential ways of slowing down or halting the condition by switching off the faulty gene that causes Huntington's disease, but there remains no cure. The leaders of this new five year UK DRI research programme are internationally recognised experts in Huntingdon’s research and the programme will open up previously unknown avenues of systematic molecular research, for real impact on our understanding of the disease.
Based on statistically robust genetic evidence, this research is focused on two very interesting Genome Wide Association Study hits implicated in repeat instability, discovered through significant contributions by the investigators. This new study aims to determine what role the molecules MSH3 and FAN1 play in Huntington’s disease progression. The researchers will also explore the role of other DNA damage response proteins that might be relevant for other neurodegenerative disorders.
The researchers have chosen high quality collaborations to bring together public and private expertise and have assembled a compelling range of cell and animal model systems upon which the work will be based. The study has a high potential to deliver important mechanistic insights and a novel therapeutic approach to Huntington’s disease.