FTD is the second most common form of young-onset dementia after Alzheimer’s disease. The disease is caused when nerve cells in the frontal and/or temporal lobes of the brain die and the pathways connecting them change. In the majority of cases, the disease is not inherited.
Professor John Hardy of the NIHR Queen Square Dementia Biomedical Research Unit, who co-led the study, said: “We are trying to understand what causes or predisposes people to the non-inherited form of FTD. Even though the disease is sporadic (not inherited), there are genetic pre-dispositions. This study is the type where we can find those pre-disposing factors.”
The study, published in Lancet Neurology, involved more than 40 research groups from Europe, Australia and the US who contributed several thousand FTD cases for analysis.
The study found evidence of two key processes driving FTD development. Professor Hardy said: “The first is an out-of-control inflammatory process in the brain and the second is a lysosomal problem. Lysosomes are parts of the cell which are responsible for digesting damaged proteins. What we think this is telling us is that these damaged proteins are overwhelming the damage response part of the cell.”
This finding in FTD raises interesting parallels with Parkinson’s Disease, another neurodegenerative disease that has been linked to similar processes.
Lead author Dr Raffaele Ferrari (UCL Institute of Neurology) said: “These findings provide the basis for future genetic and functional studies aimed at replicating these results, as well as shedding light on the molecular mechanisms that contribute to the pathogenesis of the disease.”
Professor Martin Rossor, Director of the NIHR Queen Square Dementia Biomedical Research Unit and NIHR National Research Dementia Professor said: “The study shows the value of international collaboration to amass the data needed. It also shows how much can be learned from one neurodegenerative disease which is of value to understanding others.”
- Research paper in The Lancet
- Professor John Hardy’s academic profile on Iris
- UCL Faculty of Brain Sciences
- UCL Institute of Neurology
- National Institute for Health Research University College London Hospitals Biomedical Research Centre
- Diagram showing areas of the brain affected by FTD