MOUSE SOD1 AND HUMAN/MOUSE SOD1 CHIMERAS

小鼠 SOD1 和人/小鼠 SOD1 嵌合体

• 批准号: 8361708
• 负责人: Peter JOHN HART
• 金额: $ 1.1万
• 依托单位: CORNELL UNIVERSITY
• 依托单位国家: 美国
• 财政年份: 2011
• 资助国家: 美国
• 起止时间: 2011-04-01 至 2012-03-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/8361708
• 关键词: Amyotrophic Lateral Sclerosis; Bees; Beryllium; Chimera organism; Enzymes; Funding; Grant; Human; Indium; Inherited; Link; Mus; Mutation; National Center for Research Resources; Principal Investigator; Protein S; Proteins; Relative (related person); Research; Research Infrastructure; Resources; Source; Spinal Cord; Transgenic Mice; United States National Institutes of Health; copper zinc superoxide dismutase; cost; mutant; structural biology

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. Primary support for the subproject and the subproject's principal investigator may have been provided by other sources, including other NIH sources. The Total Cost listed for the subproject likely represents the estimated amount of Center infrastructure utilized by the subproject, not direct funding provided by the NCRR grant to the subproject or subproject staff. Mutations in human copper-zinc superoxide dismutase (SOD1) are linked to an inherited form of amyotrophic lateral sclerosis (ALS). Spinal cords of transgenic mice expressing these human S proteins contain inclusions of the human mutant proteins, but endogenous mouse SOD1 is not f as a component of these aggregates. Two sequence elements in the human enzyme have bee identified that seem to enhance its aggregation relative to the mouse enzyme. These sequence elements are under study as part of SOD1 chimeras containing mouse and human sequences.

该子项目是利用资源的众多研究子项目之一 由 NIH/NCRR 资助的中心拨款提供。子项目的主要支持 并且子项目的主要研究者可能是由其他来源提供的， 包括其他 NIH 来源。 子项目可能列出的总成本 代表子项目使用的中心基础设施的估计数量， NCRR 赠款不直接向子项目或子项目工作人员提供资金。 人类铜锌超氧化物歧化酶 (SOD1) 的突变与遗传性肌萎缩侧索硬化症 (ALS) 相关。表达这些人类 S 蛋白的转基因小鼠的脊髓含有人类突变蛋白的内含物，但内源性小鼠 SOD1 并不是这些聚集体的组成部分。已鉴定出人类酶中的两个序列元件似乎相对于小鼠酶增强了其聚集。 这些序列元件作为包含小鼠和人类序列的 SOD1 嵌合体的一部分正在研究中。