Studies of a novel manganese transporter in lysosomes and its implications in Niemann-Pick type-C disease

溶酶体中新型锰转运蛋白的研究及其对尼曼-皮克 C 型疾病的影响

• 批准号: nhmrc : 1007427
• 负责人: Dr He Li
• 金额: $ 35.14万
• 依托单位: Murdoch Childrens Research Institute
• 依托单位国家: 澳大利亚
• 项目类别: NHMRC Project Grants
• 财政年份: 2011
• 资助国家: 澳大利亚
• 起止时间: 2011-01-01 至 2013-12-31
• 项目状态: 已结题
• 来源: https://researchdata.edu.au/studies-novel-manganese-c-disease/109217
• 关键词: Studies; novel; manganese; transporter; lysosomes

Niemann-Pick type-C disease is a devastating disease affecting child central nervous system. It is due to too much build up of cholesterol in the lysosomal compartment of cells. Recent studies indicate that bivalent cations are involved in the development of the disease. We recently discovered a manganese transporter in the lysosomal compartment and its interaction of the a protein that causing the disease. We will elucidate how manganese transporter controls trace metal balance and may prevent the disease.

C型尼曼-匹克病是一种严重影响儿童中枢神经系统的疾病。这是由于过多的胆固醇积聚在细胞的溶酶体区室。最近的研究表明，二价阳离子参与了疾病的发展。我们最近发现了一个锰转运蛋白在溶酶体区室和它的相互作用的蛋白质，导致疾病。我们将阐明锰转运蛋白如何控制微量金属平衡，并可能预防疾病。