Mechanisms of Toxicity in Huntington's Disease
Huntington's disease is caused by dominant mutations in the Huntingtin gene. The mutations lead the gene product, Huntingtin, to aggregate into visible inclusion bodies in neurons. We are interested in understanding the molecular basis for why the mutant proteins are toxic and how this relates to the physical process of aggregation. We study cellular models of Huntington disease and apply a broad range of methods to measure the toxicity of the mutant Huntingtin gene product.
Our team has developed novel methods to measure precisely how the protein aggregates and tp separate cells that have the mutant protein aggregated at different stages of the aggregation process. We are studying the changes in the cells as the protein aggregates.
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