HUNTINGTIN MICROAGGREGATES AND CELL TOXICITY: LIVE CELL IMAGING

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。列出的机构是 中心，不一定是研究者的机构。 亨廷顿包涵体是在亨廷顿病患者死后发现的。然而，先前显示亨廷顿蛋白包涵体形成降低神经元死亡的风险。我们的假设是，亨廷顿蛋白微聚集体负责神经毒性，并可以作为治疗干预的目标。为了验证这一假设，我们在神经元细胞系中表达带有四半胱氨酸标签的亨廷顿蛋白外显子1，并使用活细胞成像来量化可溶性亨廷顿蛋白转化为聚集体及其对细胞毒性的影响。

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.