MUTANT SOD1 ASSOCIATION WITH MITOCHONDRIA

突变体 SOD1 与线粒体的关联

• 批准号: 8365861
• 负责人: Don W Cleveland
• 金额: $ 0.74万
• 依托单位: UNIVERSITY OF WASHINGTON
• 依托单位国家: 美国
• 财政年份: 2011
• 资助国家: 美国
• 起止时间: 2011-09-01 至 2012-06-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/8365861
• 关键词: Amyotrophic Lateral Sclerosis; Biology; Familial Amyotrophic Lateral Sclerosis; Funding; Fungal Genome; Grant; Liver; Mass Spectrum Analysis; Mediating; Mitochondria; Mutation; National Center for Research Resources; Phenotype; Principal Investigator; Proteins; Rattus; Research; Research Infrastructure; Resources; Source; Spinal Cord; United States National Institutes of Health; cost; mutant; superoxide dismutase 1

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 superoxide dismutase 1 (SOD1) cause familial amyotrophic lateral sclerosis (ALS). Expression of mutant SOD1 (SOD1G93A) in rats causes an ALS-like phenotype. Mutant SOD1 associates with mitochondria from the spinal cord, but not the liver. The proteins that mediate the interaction of SOD1 with mitochondria are unknown. We have immunoprecipitated SOD1 from spinal cord mitochondrial fractions and propose to identify proteins associated with SOD1 by mass spectrometry.

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。 超氧化物歧化酶 1 (SOD1) 突变会导致家族性肌萎缩侧索硬化症 (ALS)。 突变型 SOD1 (SOD1G93A) 在大鼠中的表达会导致 ALS 样表型。 突变型 SOD1 与脊髓线粒体相关，但与肝脏线粒体无关。 介导 SOD1 与线粒体相互作用的蛋白质尚不清楚。 我们从脊髓线粒体组分中免疫沉淀 SOD1，并建议通过质谱法鉴定与 SOD1 相关的蛋白质。