Tammaryn Lashley
Alzheimer's Research UK
United Kingdom
The role of the nuclear transport system in frontotemporal dementia.
Alzheimer's Research UK
231,465
01/02/13
3.3
Alzheimer's disease & other dementias
Brain Circuits and Synapses | Selective Vulnerability
Frontotemporal lobar degenerations (FTLDs) are collectively the second most common form of young onset dementia, characterised by progressive nerve cell loss in the frontal and temporal lobes, and neuropathological hallmarks formed from proteins (TAR-DNA binding protein (TDP-43) and fused in sarcoma (FUS)) becoming defective. The normal function of the TDP-43 and FUS proteins are involved in the movement of ribonucleic acid (RNA, genetic information which directs the production of proteins) from the cell nucleus (control centre) to the outer part of the cell, the cytoplasm. We have shown for the first time that additional proteins involved in the movement of RNA are also found in the neuropathological hallmarks of FTLDs, suggesting these may play a role in the disease progression. Utilising our unique collection of post mortem FTLD cases this study aims to identify brain areas that are vulnerable to developing pathology and compare them to areas that are pathologically unharmed. By identifying these additional proteins and the changes in protein levels we hope to highlight pathological events occurring early in the development of FTLDs, which potentially could be targeted for early therapeutic interventions to prevent or slow the progression of the disease.