The Role of Huntingtin Misfolding and Oligomerization in Huntingtons Disease

亨廷顿蛋白错误折叠和寡聚化在亨廷顿病中的作用

• 批准号: nhmrc : 566640
• 负责人: A/Pr Daniel Hatters
• 金额: $ 31.63万
• 依托单位: The University of Melbourne
• 依托单位国家: 澳大利亚
• 项目类别: NHMRC Project Grants
• 财政年份: 2009
• 资助国家: 澳大利亚
• 起止时间: 2009-01-01 至 2012-12-31
• 项目状态: 已结题
• 来源: https://researchdata.edu.au/role-huntingtin-misfolding-huntingtons-disease/81925
• 关键词: Role; Huntingtin; Misfolding; Oligomerization; Huntingtons

Mutations in the huntingtin gene cause Huntington's disease by making the gene product aggregate together into non-normal and different sized polymers. However, it is not understood how this process causes cells to die, largely because we don't understand how the abnormal forms accumulate in cells over time. We will examine where in cells the abnormal shapes accumulate and how they cause toxicity. This research will identify critically-needed therapeutic targets against Huntington's disease.

亨廷顿基因的突变导致亨廷顿氏病，使基因产物聚集在一起成为不正常的和不同大小的聚合物。然而，目前还不清楚这一过程是如何导致细胞死亡的，主要是因为我们不了解异常形式如何随着时间的推移在细胞中积累。我们将研究异常形状在细胞中的何处积聚以及它们如何引起毒性。这项研究将确定急需的治疗目标，对亨廷顿病。