Grant: Operating Grant

In the brain misfolded and damaged proteins accumulate with age or as the consequence of mutations in inherited disorders such as Huntington's disease. A hallmark of many forms of human neurodegenerative disease is the formation of inclusion bodies within neurons; these structures are thought to represent concentrations of misfolded proteins that have been sequestered away from other cellular constituents. We have recently shown that a protein called FUS becomes concentrated into such inclusions, and this may have deleterious effects on cell survival. We propose to study this process using live cell imaging and computer modelling. We will identify the signalling pathways that impinge on FUS and determine if these pathways can be therapeutically manipulated.