MUTANT SOD1 ASSOCIATION WITH MITOCHONDRIA

这个子项目是许多利用资源的研究子项目之一 由NIH/NCRR资助的中心赠款提供。次级项目的主要支助 而子项目的主要调查员可能是由其他来源提供的， 包括其它NIH来源。 为子项目列出的总成本可能 代表子项目使用的中心基础设施的估计数量， 而不是由NCRR赠款提供给子项目或子项目工作人员的直接资金。 超氧化物歧化酶1（SOD 1）突变导致家族性肌萎缩侧索硬化症（ALS）。 突变体SOD 1（SOD 1G 93 A）在大鼠中的表达导致ALS样表型。 突变的SOD 1与来自脊髓的线粒体相关，但不与肝脏相关。 介导SOD 1与线粒体相互作用的蛋白质是未知的。 我们免疫沉淀SOD 1从脊髓线粒体组分，并建议通过质谱鉴定与SOD 1相关的蛋白质。

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.