QBI Neuroscience Seminar: “Mechanisms of neurodegeneration and neuroprotection in motor neuron disease and frontotemporal dementia”
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- Dr Adam Walker
Queensland Brain Institute
University of Queensland
Title: “Mechanisms of neurodegeneration and neuroprotection in motor neuron disease and frontotemporal dementia”
Abstract: Pathology formed by the RNA-binding protein TDP-43 is the primary hallmark of disease in almost all cases of motor neuron disease (MND) and approximately half of all cases of frontotemporal dementia (FTD). In disease, TDP-43 relocates from the nucleus and accumulates in the cytoplasm, where it is extensively post-translationally modified and aggregates to form characteristic intracellular inclusions. There is remarkable heterogeneity in the structure and distribution of TDP-43 inclusions in disease, and how TDP-43 aggregation causes neurodegeneration remains unclear. In this presentation, I will discuss recent studies in my laboratory using cell and mouse models of TDP-43 pathology coupled with biochemistry, proteomics and whole-genome CRISPR screening approaches aimed towards identifying mechanisms involved in TDP-43-associated neurodegeneration and characterising potential targets for future therapeutic development for people living with MND and FTD.
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