Altered nuclear trafficking and nuclear body dynamics as drivers of ataxin-1 toxicity

改变核运输和核体动力学作为 ataxin-1 毒性的驱动因素

• 批准号: nhmrc : GNT1121907
• 负责人: A/Pr Daniel Hatters
• 金额: $ 75.52万
• 依托单位国家: 澳大利亚
• 项目类别: Project Grants
• 财政年份: 2017
• 资助国家: 澳大利亚
• 起止时间: 2017-01-01 至 无数据
• 项目状态: 已结题
• 来源: https://researchdata.edu.au/altered-nuclear-trafficking-1-toxicity/1347235
• 关键词: Altered; nuclear; trafficking; body; dynamics

Ataxias are a large group of neurodegenerative disorders in which balance, motor skills and memory are progressively lost. While mutations in specific proteins do cause certain hereditary ataxias, the mechanisms of their detrimental actions is unclear. Our studies probe the toxic mechanisms of the ataxin-1 protein, focusing on its partners and stress-initiated formation of a toxic hydrogel state. The outcomes will define impacts on cellular protein movement in neurodegeneration more broadly.

共济失调是一大类神经退行性疾病，其中平衡、运动技能和记忆逐渐丧失。虽然特定蛋白质的突变确实会导致某些遗传性共济失调，但其有害作用的机制尚不清楚。我们的研究探讨了共济失调蛋白-1蛋白的毒性机制，重点是它的伙伴和压力引发的有毒水凝胶状态的形成。这些结果将更广泛地定义神经变性对细胞蛋白质运动的影响。