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Immune system implicated in dementia development

18 June 2014

The immune system and body’s response to damaged cells play a key role in the development of frontotemporal dementia (FTD), finds new research led by the UCL Institute of Neurology.

Diagram showing areas of the brain affected by FTD

These findings will enable researchers to develop new treatments that target the body’s response to damaged cells. 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.

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.

Professor John Hardy, co-leader of the study, NIHR Queen Square Dementia Biomedical Research Unit

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.

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.

Professor John Hardy

This finding in FTD raises interesting parallels with Parkinson’s Disease, another neurodegenerative disease that has been linked to similar processes.

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

Lead author Dr Raffaele Ferrari

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.

Professor Martin Rossor, Director of the NIHR Queen Square Dementia Biomedical Research Unit and NIHR National Research Dementia Professor

Further information:

Image

  • Diagram showing areas of the brain affected by FTD

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