- Frontotemporal dementia mostly affects people aged 45-65, although it can also affect younger or older people, causing personality, behavioural and language changes. There is currently no treatment available to cure or slow the progression of the disease.
- The funding from Alzheimer’s Research UK comes during Dementia Action Week (16-20 May) as part of a £2 million package of new research funding across the UK.
- Researchers Dr Matthew Livesey and Dr Ryan West at the University of Sheffield aim to shed more light on how frontotemporal dementia develops, which could lead to the development of new treatments.
Many factors contribute to someone’s risk of developing FTD, with genetics playing a key role. The most common genetic cause of FTD is a change, called a mutation, in a gene known as C9ORF72. This mutation causes proteins called ‘dipeptide-repeats’ to clump up in the brain, ultimately leading to a loss of brain nerve cells.
Researchers believe that this build up may disrupt the normal electrical activity of nerve cells, causing them to stop functioning and impacting the way our brain is able to perceive things and function. Yet exactly how they do this remains unclear.
In order to better understand how this happens, Sheffield researchers will investigate the physical structure and health of nerve cells in fruit flies that produce these dipeptide repeats and observe the cells’ electrical signals. They will then compare this to people living with FTD.
Dr Matthew Livesey from Department of Neuroscience at the University of Sheffield said:
“This Alzheimer’s Research UK pilot project aims to give insight into what physiological processes are disrupted by the build-up of toxic repeats sequences in the brain.
“Fruit flies are well suited for this type of study as around 75% of the disease-causing genes in people are also found in flies. Their short life span also means we can look at how the activity of genes change over time as the flies age, speeding up the research process.
“This will increase our understanding of the causes of frontotemporal dementia and could identify potential new targets for treatments.”
Professor Dame Pamela Shaw, Director of the Sheffield Institute for Translational Neuroscience (SITraN) and NIHR Sheffield Biomedical Research Centre (BRC) said:
“Changes in the C9orf72 gene represent the most frequent known cause of fronto-temporal dementia (FTD) and also motor neuron disease (MND) and the two conditions often occur together. At present no effective treatment has been found for these conditions. Dr Livesey and Dr West have an elegant fruit fly model of FTD/MND which will allow them to identify the mechanisms of nerve cell injury.
At the NIHR Sheffield Biomedical Research Centre, we have systematically collected biosamples from patients that provide a powerful way to validate laboratory findings from disease models to ensure that new targets for treatments are relevant to patients. We are then poised to be able to quickly take promising new treatments from the SITraN laboratories into experimental clinical trials.”
Dr Rosa Sancho, Head of Research at Alzheimer’s Research UK, said:
“Dementia affects nearly one million people in the UK including around 60,000 in Yorkshire alone. The condition is not an inevitable part of getting older but the result of diseases that damage the brain. Pioneering research underway in Sheffield is helping to unpick the complex causes of frontotemporal dementia and driving progress towards new treatments for people living with the condition.”
The research will take place at SITraN which is one of the world’s leading centres for research into neurodegenerative diseases including Alzheimer’s, Motor Neurone Disease and Parkinson’s Disease.