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Nature子刊:武汉大学宋质银教授发表线粒体研究文章

摘要 : 2016年3月,国际学术权威刊物自然出版集团旗下子刊《Cell Death & Disease》在线发表武汉大学生命科学学院宋质银教授课题组题为“Mic60/Mitofilin determines MICOS assembly essential for mitochondrial dynamics and mtDNA nucleoid organization ”的研究论文。

 2016年3月,国际学术权威刊物自然出版集团旗下子刊《Cell Death & Disease》在线发表武汉大学生命科学学院宋质银教授课题组题为“Mic60/Mitofilin determines MICOS assembly essential for mitochondrial dynamics and mtDNA nucleoid organization ”的研究论文。研究论文阐述了Mic60在维持MICOS复合物稳定性及其在线粒体动态调控中的重要作用。博士生李荟晖为论文的第一作者,宋质银教授为论文通讯作者。

线粒体是细胞能量产生的主要场所,又称细胞的“动力工厂”,而线粒体内膜结构嵴则是线粒体电子传递及能量(ATP)产生的关键部位。如果线粒体嵴出现异常,线粒体功能将会受损,也会引起肿瘤、心血管和神经退行性等多种线粒体相关人类疾病。该论文研究结果鉴定和发现了MICOS亚组分蛋白Mic60/Mitofilin是线粒体形态调控的关键蛋白,Mic60/Mitofilin缺失导致“巨大球型线粒体”(此类型线粒体只在衰老及一些疾病组织细胞中被发现)的形成。论文阐明了调控线粒体内膜结构嵴形态的关键复合物MICOS在哺乳动物细胞中的组装模式及分子机制,对于线粒体嵴形成及形态调控机制具有重要的理论基础和科学价值,并为衰老及线粒体相关疾病病理机制的阐明提供了新的方向。

原文链接:

Mic60/Mitofilin determines MICOS assembly essential for mitochondrial dynamics and mtDNA nucleoid organization

原文摘要:

The MICOS complex (mitochondrial contact site and cristae organizing system) is essential for mitochondrial inner membrane organization and mitochondrial membrane contacts, however, the molecular regulation of MICOS assembly and the physiological functions of MICOS in mammals remain obscure. Here, we report that Mic60/Mitofilin has a critical role in the MICOS assembly, which determines the mitochondrial morphology and mitochondrial DNA (mtDNA) organization. The downregulation of Mic60/Mitofilin or Mic19/CHCHD3 results in instability of other MICOS components, disassembly of MICOS complex and disorganized mitochondrial cristae. We show that there exists direct interaction between Mic60/Mitofilin and Mic19/CHCHD3, which is crucial for their stabilization in mammals. importantly, we identified that the mitochondrial i-AAA protease Yme1L regulates Mic60/Mitofilin homeostasis. Impaired MICOS assembly causes the formation of 'giant mitochondria' because of dysregulated mitochondrial fusion and fission. Also, mtDNA nucleoids are disorganized and clustered in these giant mitochondria in which mtDNA transcription is attenuated because of remarkable downregulation of some key mtDNA nucleoid-associated proteins. Together, these findings demonstrate that Mic60/Mitofilin homeostasis regulated by Yme1L is central to the MICOS assembly, which is required for maintenance of mitochondrial morphology and organization of mtDNA nucleoids.

来源: Cell Death & Disease 浏览次数:0

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