Ribosome. The structure of the human mitochondrial ribosome.
Ribosome. The structure of the human mitochondrial ribosome.
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DOI：
10.1126/science.aaa1193
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发表时间：
2015-04-03
期刊：
影响因子：
--
通讯作者：
Ramakrishnan V
中科院分区：
文献类型：
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作者：
Amunts A;Brown A;Toots J;Scheres SHW;Ramakrishnan V
人线粒体的高度分化的核糖体（mitoribosomes）合成氧化磷酸化复合物的13种必需蛋白质。我们通过单粒子冷冻电子显微镜以3.5埃的分辨率确定了完整线粒体的结构。它揭示了80种广泛相互连接的蛋白质，其中36种是线粒体特有的，还有3种rRNA分子。小亚基的头部结构域，特别是mRNA通道，高度重塑。许多亚基间桥是线粒体所特有的，它采用的构象涉及棘轮或滚动的小亚基，这是不同于那些在细菌或真核生物中看到的。内在的GTPase介导头部和中央突起之间的接触。该结构为分析导致严重病理的突变和未来的药物设计提供了参考。
The highly divergent ribosomes of human mitochondria (mitoribosomes) synthesize 13 essential proteins of oxidative phosphorylation complexes. We have determined the structure of the intact mitoribosome to 3.5 angstrom resolution by single-particle electron cryo-microscopy. It reveals 80 extensively interconnected proteins, 36 of which are specific to mitochondria, and three rRNA molecules. The head domain of the small subunit, particularly the mRNA channel, is highly remodeled. Many intersubunit bridges are unique to the mitoribosome, which adopts conformations involving ratcheting or rolling of the small subunit that are distinct from those seen in bacteria or eukaryotes. An intrinsic GTPase mediates a contact between the head and central protuberance. The structure provides a reference for analysis of mutations that cause severe pathologies, and for future drug design.