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Importance of copper chaperone Cox17p in early embryogenesis.

铜伴侣 Cox17p 在早期胚胎发生中的重要性。

基本信息

批准号：
15370050
负责人：
KAKO Koichiro
金额：
$ 4.16万
依托单位：
University of Tsukuba
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (B)
财政年份：
2003
资助国家：
日本
起止时间：
2003 至 2004
项目状态：
已结题

来源：
https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-15370050/
关键词：
Cox17p mitochondria cytochrome c oxidase Sco1 2p central nervous system in situ hybridization 細胞内銅輸送機構

项目摘要

It is well known that the deficiencies in cytochrome c oxidase (CCO) are relatively rare but most often lethal. CCO, one of the mitochodrial respiratory enzymes, requires Cu (I) to express its enzymatic activity. Cox17p, Scolp, and its homologue Sco2p are thought to be involved in mitochondrial transport of copper to CCO. By in vitro GST-pull down assay, both Sco1p and Sco2p interact with Cox17p. Furthermore, Cox17p, Scolp and Sco2p also interact with Cox2p, a subunit of the binuclear-copper centre Cu_A of CCO. Northern blot analysis demonstrated that Cox17p, Sco1p and Sco2p are widely expressed at varying levels in a variety of mouse tissues including brain. Using in situ hybridization histochemistry, we characterized the expression profiles for these molecules in different regions of the normal adult mouse brain. The highest levels of these messages were associated with distinct neuronal subtypes, including pyramidal cell layers of hippocampus and ventral cochlear nucleus of pons, that were also contained high levels of CCO activity. Although CCO activities and expression levels of Cox17p and Scolp were relatively high, the expression of Sco2p was quite low in some subsets of neurons such as cerebellum Purkinje cells. It was thought that the difference of expression profile between Sco1p and Sco2p is originated in that of genomic structures, especially the promoter regions.

众所周知，细胞色素c氧化酶（CCO）的缺陷是相对罕见的，但最常见的是致命的。CCO是线粒体呼吸酶之一，需要Cu（I）来表达其酶活性。Cox 17 p、Scolp及其同源物Sco 2 p被认为参与铜向CCO的线粒体转运。通过体外GST-下拉分析，Sco 1 p和Sco 2 p都与Cox 17 p相互作用。Cox 17 p、Scolp和Sco 2 p还与CCO双核铜中心Cu_A的亚基Cox 2 p相互作用。北方印迹分析表明Cox 17 p、Sco 1 p和Sco 2 p在包括脑在内的多种小鼠组织中以不同水平广泛表达。使用原位杂交组织化学，我们的特点是这些分子在正常成年小鼠大脑的不同区域的表达谱。这些信息的最高水平与不同的神经元亚型相关，包括海马锥体细胞层和脑桥腹侧耳蜗核，它们也含有高水平的CCO活性。虽然CCO活性和Cox 17 p和Scolp的表达水平相对较高，但在小脑浦肯野细胞等某些神经元亚群中，Sco 2 p的表达相当低。人们认为Sco 1 p和Sco 2 p表达谱的差异源于基因组结构的差异，尤其是启动子区的差异。