Caenorhabditis elegans Mitofilin Homologs Control the Morphology of Mitochondrial Cristae and Influence Reproduction and Physiology
Caenorhabditis elegans Mitofilin Homologs Control the Morphology of Mitochondrial Cristae and Influence Reproduction and Physiology
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DOI：
10.1002/jcp.22177
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发表时间：
2010-09-01
影响因子：
5.6
通讯作者：
Han, Sung Sik
中科院分区：
文献类型：
--
作者：
Mun, Ji Young;Lee, Tae Hoon;Han, Sung Sik
人丝裂素是一种控制嵴形成的线粒体蛋白。在这里，我们调查的作用，秀丽隐杆线虫mitofilin同系物，IMMT-1和-2，在生殖，生理和线粒体嵴的形成。immt-1或immt-2的突变导致生殖系发育和产卵缺陷。这些缺陷被双重突变所加剧，这大大降低了运动性，增加了活性氧的水平，降低了线粒体质量，并赋予了对氧化应激的抵抗力。冷冻电子显微镜和电子断层扫描显示，每个单一的突变导致弯曲和堆叠的线粒体嵴小管以及嵴连接的数量减少。immt-2突变也与线粒体外膜孔的存在有关，在双突变体中这些孔更大。IMMT-1和IMMT-2蛋白定位于线粒体内膜，如通过免疫电镜所见，并且它们在天然聚丙烯酰胺凝胶电泳中表现为与F1 F0 ATP合酶的寡聚体或大复合物。这些结果表明，两个C。秀丽线虫丝裂蛋白同种型在控制线粒体嵴形成中具有非重叠的功能。J.细胞。Physiol.224：748- 756，2010。(C)2010 Wiley-Liss，Inc.
Human mitofilin is a mitochondrial protein that controls cristae formation. Here, we investigated the role of the Caenorhabditis elegans mitofilin homologs, IMMT-1 and -2, in reproduction, physiology, and mitochondrial cristae formation. Mutation of either immt-1 or immt-2 produced defects in germline development and egg-laying. These defects were exacerbated by the double mutation, which greatly reduced motility, increased levels of reactive oxygen species, decreased mitochondrial mass, and imparted resistance to oxidative stress. Cryo-electron microscopy and electron tomography revealed that each of the single mutations resulted in curved and stacked mitochondrial crista tubules as well as a reduced number of crista junctions. The immt-2 mutation was also associated with the presence of outer mitochondrial membrane pores, which were larger in the double mutant. IMMT-1 and IMMT-2 proteins were localized to the inner mitochondrial membrane, as seen by immunoelectron microscopy, and they behaved as oligomers or large complexes with F1F0 ATP synthase in native polyacrylamide gel electrophoresis. These findings suggest that the two C. elegans mitofilin isoforms have non-overlapping functions in controlling mitochondrial cristae formation. J. Cell. Physiol.224: 748-756,2010. (C) 2010 Wiley-Liss, Inc.