Characterization of transcriptionally regulated genes

线粒体是真核生物必不可少的复杂细胞器。 各种代谢过程所需的有机体，包括 通过氧化磷酸化产生能量。生物发生与维护 线粒体的功能需要分子伴侣的功能，如Hsp7O。 我们的长期目标是了解分子的作用机制。 以酿酒酵母为模型系统的线粒体中的伴侣。 利用遗传学和生物化学的方法，分析 Hsp7O和Hsp4O类线粒体伴侣蛋白在血管紧张素转换酶损伤中的作用 线粒体蛋白的蛋白质易位、折叠和组装 继续。这些研究与人类健康问题有关，可以肯定的是 人体组织，如大脑、心脏、肌肉和肾脏，尤其是 依赖于有效的线粒体功能。引起的病理影响 生物能能力的降低已被发现是由两者的突变引起的。 人类种群中的线粒体和人类DNA。此外，其中一项 线粒体Hsp7os与Hsp7os同源物的成熟有关 与神经退行性疾病有关的人类Frataxin 弗雷德里希共济失调。 Ssc1是线粒体基质中的一种Hsp7O，是 从胞质中转运蛋白质所需的装置。SSC 1是 通过其与基本外围设备的交互被拴在进口通道上 通道的组件Tim44。MGE-1，一种必需的核苷酸释放因子 对于SSC 1，还与导入通道上的SSC1相关联。Hsp4O的SSC1 Mdj1和核苷酸交换因子mGel被认为促进折叠 进口蛋白质。这项提议旨在理解 Ssc1在蛋白质跨线粒体蛋白转运中的作用 它们随后在基质中折叠。 SSQ1和Jaci分别是的附加Hsp7O和Hsp4O 线粒体基质。SSQ1在铁代谢调节中的作用 和/或指示了Fe-S中心的组装。我们的目标是了解 SSQ1在线粒体中的功能(S)并提供对细胞 SQL执行操作的进程。缺乏的主要和次要影响 SSQL的功能，重点是铁的代谢，包括它在 酵母Frataxin同系物YFH_1的成熟及Fe-S的组装 集群将使用遗传和生化的组合来确定 技巧。

Mitochondria are essential, complex organelles of eucaryotic organisms required for a variety of metabolic processes including the generation of energy by oxidative phosphorylation. Biogenesis and maintenance of mitochondria requires the function of molecular chaperones such as Hsp7O. Our long term goal is to understand the mechanism of action of molecular chaperones within mitochondria using S. cerevisiae as a model system. Using genetic and biochemical approaches the analysis of the roles of mitochondrial chaperones of the Hsp7O and Hsp4O classes in the processes of protein translocation, folding and assembly of mitochondrial proteins will be continued. These studies are relevant to issues of human health, as certain human tissues, such as brain, heart, muscle and kidney, are particularly dependent on efficient mitochondrial function. Pathological effects caused by reduced bioenergetic capacity have been found to be caused by mutations in both mitochondrial and human DNA in human populations. In addition, one of the mitochondrial Hsp7Os has been implicated in the maturation of the homologue of human frataxin, which is associated with the neurodegenerative disease Freidrich's ataxia. Ssc1, an Hsp7O of the mitochondrial matrix, is an essential component of the apparatus required for translocation of proteins from the cytosol. Ssc 1 is tethered to the import channel via its interaction with an essential peripheral component of the channel, Tim44. Mge 1, an essential nucleotide release factor for Ssc 1, is also associated with Ssc1 at the import channel. Ssc1, an Hsp4O Mdjl, and the nucleotide exchange factor Mgel are thought to facilitate folding of imported proteins. This proposal is designed to understand the pathway of Ssc1 function in translocation of proteins across the mitochondnal proteins and their subsequent folding in the matrix. Ssq1 and Jaci are additional Hsp7Os and Hsp4Os, respectively, of the mitochondrial matrix. A role for Ssq1 in the regulation of iron metabolism and/or the assembly of Fe-S centers is indicated. Our goal is to understand the function(s) of Ssq1 in mitochondria and to provide insight into the cellular process(es) in which Ssql acts. The primary and secondary effects of the lack of Ssql function, focusing on iron metabolism, including its role in the maturation of Yfh 1, the yeast homologue of frataxin, and assembly of Fe-S clusters will be determined using a combination of genetic and biochemical techniques.