MPV17L2 is required for ribosome assembly in mitochondria.
MPV17L2 is required for ribosome assembly in mitochondria.
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DOI：
10.1093/nar/gku513
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发表时间：
2014-07
影响因子：
14.9
通讯作者：
Spinazzola A
中科院分区：
文献类型：
--
作者：
Dalla Rosa I;Durigon R;Pearce SF;Rorbach J;Hirst EM;Vidoni S;Reyes A;Brea-Calvo G;Minczuk M;Woellhaf MW;Herrmann JM;Huynen MA;Holt IJ;Spinazzola A
MPV17是一种功能未知的线粒体蛋白，MPV17的突变与线粒体脱氧核糖核酸（DNA）维持障碍有关。在这里，我们研究了它最相似的亲戚MPV17L2，它也被注释为线粒体蛋白。线粒体分离分析表明MPV17L2和MPV17一样是一个完整的内膜蛋白。然而，与MPV17不同的是，MPV17L2依赖于线粒体DNA，因为它不存在于ρ0细胞中，并且与线粒体核糖体和单体的大亚基共同沉积在蔗糖梯度上。MPV17L2基因沉默导致线粒体核糖体的单体和两个亚基显著减少，导致线粒体中蛋白质合成受损。MPV17L2的缺失也会诱导线粒体DNA聚集。DNA和核糖体表型是相关联的，因为在缺乏MPV17L2的情况下，线粒体核糖体的小亚基蛋白被困在增大的类核中，而大亚基的组成部分则相反。这些发现表明MPV17L2有助于线粒体核糖体的生物发生，将两个亚基联合起来形成具有翻译能力的单体，并提供证据表明线粒体核糖体小亚基的组装发生在类核中。
MPV17 is a mitochondrial protein of unknown function, and mutations in MPV17 are associated with mitochondrial deoxyribonucleic acid (DNA) maintenance disorders. Here we investigated its most similar relative, MPV17L2, which is also annotated as a mitochondrial protein. Mitochondrial fractionation analyses demonstrate MPV17L2 is an integral inner membrane protein, like MPV17. However, unlike MPV17, MPV17L2 is dependent on mitochondrial DNA, as it is absent from ρ0 cells, and co-sediments on sucrose gradients with the large subunit of the mitochondrial ribosome and the monosome. Gene silencing of MPV17L2 results in marked decreases in the monosome and both subunits of the mitochondrial ribosome, leading to impaired protein synthesis in the mitochondria. Depletion of MPV17L2 also induces mitochondrial DNA aggregation. The DNA and ribosome phenotypes are linked, as in the absence of MPV17L2 proteins of the small subunit of the mitochondrial ribosome are trapped in the enlarged nucleoids, in contrast to a component of the large subunit. These findings suggest MPV17L2 contributes to the biogenesis of the mitochondrial ribosome, uniting the two subunits to create the translationally competent monosome, and provide evidence that assembly of the small subunit of the mitochondrial ribosome occurs at the nucleoid.
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DOI：
10.1083/jcb.93.1.97
发表时间：
1982-04
期刊：
The Journal of cell biology
影响因子：
--
作者：
Fujiki Y;Hubbard AL;Fowler S;Lazarow PB
通讯作者：
Lazarow PB
DOI：
10.1042/bj20100757
发表时间：
2010-09-15
期刊：
The Biochemical journal
影响因子：
--
作者：
Dennerlein S;Rozanska A;Wydro M;Chrzanowska-Lightowlers ZM;Lightowlers RN
通讯作者：
Lightowlers RN
影响因子：
64.8
作者：
Greber, Basil J.;Boehringer, Daniel;Ban, Nenad
通讯作者：
Ban, Nenad
影响因子：
3.5
作者：
Dallabona, Cristina;Marsano, Rene Massimiliano;Donnini, Claudia
通讯作者：
Donnini, Claudia
影响因子：
3.5
作者：
Hasegawa, Ayako;van der Bliek, Alexander M.
通讯作者：
van der Bliek, Alexander M.