HUNTINGTIN MICROAGGREGATES AND CELL TOXICITY: LIVE CELL IMAGING

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 亨廷顿S病患者死后发现亨廷顿蛋白包涵体。然而，先前的研究表明，亨廷顿蛋白包涵体的形成可以降低神经元死亡的风险。我们的假设是，亨廷顿蛋白微团聚体是神经毒性的罪魁祸首，可以作为治疗干预的靶点。为了验证这一假设，我们在神经细胞系中表达了带有四个半胱氨酸标签的Huntingtin外显子-1，并使用活细胞成像来量化可溶性Huntingtin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.