UCL Queen Square Institute of Neurology


UK DRI awards Faculty researchers £1.5M to study the DNA damage in Huntington's

18 December 2017

The UK Dementia Research Institute (UK DRI) have announced a £1.5 million award to fund research into Huntington’s disease which will be led by UCL Institute of Neurology researchers Professors Gillian Bates and Sarah Tabrizi. The programme 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 Huntington’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.

"Sarah Tabrizi and I are delighted that the UK DRI will fund our research programme aimed at understanding the mechanism by which genetic modifiers influence the age of onset and progression of Huntington’s disease and the potential for therapeutic benefit. This will embed a UK DRI research programme within our Huntington’s Disease Centre and bring our research closer to the wider UK-based dementia research community" Prof Gill Bates, UK DRI at UCL

This is the first UK DRI funded programme of work that tackles Huntingdon’s disease and showcases the type of science that will be a hallmark of the UK DRI - innovative, collaborative and world leading. UK DRI will carry out research relevant to all dementias, including Alzheimer’s disease, Parkinson’s disease, frontotemporal dementia, vascular dementia, Huntington’s disease and beyond.

"The most recent addition to the UK DRI at UCL are two famous Huntington's researchers: Gill Bates and Sarah Tabrizi. Together they oversee a broad spectrum of fantastic Huntington's research: from basic science up to clinical trials trying to modify the course of this terrible disease. They bring an exciting programme around DNA damage and repair. We are really pleased to have both on board: they bring an enormous expertise to the UK DRI and all of us can learn from the scientific insights of these two leaders in their field". Prof Bart De Strooper, UK DRI Director

Source: UK DRI website

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