Prion protein misfolding the structural biology of prion diseases

朊病毒蛋白错误折叠朊病毒疾病的结构生物学

• 批准号: BB/D005027/1
• 负责人: John Viles
• 金额: $ 48.06万
• 依托单位: Queen Mary University of London
• 依托单位国家: 英国
• 项目类别: Research Grant
• 财政年份: 2006
• 资助国家: 英国
• 起止时间: 2006 至 无数据
• 项目状态: 已结题
• 来源: https://gtr.ukri.org/projects?ref=BB%2FD005027%2F1
• 关键词: Prion; protein; misfolding; structural; biology

The proposal would fund two research workers for 3 years to address fundamental molecular processes associated with Prion diseases, which include CJD in humans and mad-cow disease in cattle. A key feature of this fatal neurodegenerative disease is the accumulation of a small molecule, the prion protein within the brain. The prion protein changes shape in the diseased form and then clumps together to form the insoluble plaques found in the brains of patients with prion disease. It is the ability of the prion protein to change shape and misfold that will be studied. The interaction of the prion protein with physiological levels of the essential mineral copper will also be investigated, looking into how for example copper influences the shape (structure) and misfolding of the prion protein. These studies will inform drug design and assist in the development of medicines for the disease. Prion diseases have similarities with the more prevalent neurodegenerative disease Alzheimer's and knowledge gained in this study will increase our understanding of both diseases.

该提案将为两名研究人员提供为期3年的资金，以解决与Prion疾病相关的基本分子过程，其中包括人类的CJD和牛的疯牛病。这种致命的神经退行性疾病的一个关键特征是大脑中积累了一种小分子，即普恩蛋白。Prion蛋白在患病状态下改变形状，然后聚集在一起，形成在Prion病患者大脑中发现的不可溶的斑块。将研究的是Pron蛋白改变形状和错误折叠的能力。还将研究Pron蛋白与必需矿物质铜的生理水平的相互作用，例如铜如何影响Pron蛋白的形状(结构)和错误折叠。这些研究将为药物设计提供信息，并帮助开发治疗这种疾病的药物。Prion病与更普遍的神经退行性疾病阿尔茨海默氏症有相似之处，这项研究获得的知识将增加我们对这两种疾病的了解。