HUNTINGTIN MICROAGGREGATES AND CELL TOXICITY: LIVE CELL IMAGING

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This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Huntingtin inclusion bodies are found postmortem in Huntington¿s disease patients. However it was previously shown that huntingtin inclusion body formation reduces the risk of neuronal death. Our hypothesis is that huntingtin microaggregates are responsible for neurotoxicity, and could serve as a target for therapeutic intervention. To test this hypothesis, we are expressing huntingtin exon-1 with a tetra cysteine tag in neuronal cell lines and using live cell imaging to quantify the conversion of soluble huntingtin into aggregates and its effect on cell toxicity.