STRUCTURE-FUNCTION STUDIES OF MITOCHONDRIA

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 该项目旨在提供线粒体结构-功能方面的初步数据，特别侧重于线粒体类核，这是一种被认为是线粒体DNA分离的基础的线粒体DNA-蛋白质结构。作为初步研究，我们将从缺乏各种线粒体成分的野生型和突变株的小鼠组织中部分纯化线粒体。我们建议检查缺乏琥珀酰辅酶A连接酶(SUCLA2)的小鼠的脑线粒体，在酵母中，琥珀酰辅酶A连接酶(SUCLA2)在酵母中被分配给类核，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也有类似的作用。第二个感兴趣的品系是缺乏VDAC(电压依赖阴离子通道)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.