STRUCTURE-FUNCTION STUDIES OF MITOCHONDRIA

该子项目是利用 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得主要资金， 因此可以在其他CRISP条目中表示。列出的机构是 中心，不一定是研究者的机构。 该项目旨在提供有关线粒体结构-功能方面的初步数据，特别关注线粒体类核，这是一种被认为是线粒体DNA分离基础的mtDNA-蛋白质结构。作为一个初步的研究，我们将部分纯化线粒体从小鼠组织中的野生型和突变株缺乏各种线粒体成分。我们建议从缺乏琥珀酰CoA连接酶（SUCLA 2）的小鼠品系中检查脑线粒体，该酶在酵母中已被分配给类核，当在人类中突变时会导致mtDNA耗尽，这表明在哺乳动物类核中具有类似的作用。第二种感兴趣的品系是缺乏VDAC（电压依赖性阴离子通道）的小鼠，VDAC是主要的通道途径。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. This project is designed to provide preliminary data on structure-function aspects of mitochondria, with a specific focus on the mitochondrial nucleoid, a mtDNA-protein structure that is considered the basis for mitochondrial DNA segregation. As a preliminary study we will partially purify mitochondria from mouse tissues in both wildtype and mutant strains lacking various mitochondrial components. We propose to examine brain mitochondria from a mouse strain that lacks succinyl CoA ligase (SUCLA2), which in yeast has been assigned to the nucleoid and when mutated in humans leads to mtDNA depletion, suggesting a similar role in mammalian nucleoids. A second strain of interest are mice lacking VDACs (Voltage-dependent Anion Channels), the main channel pathway.