Dulbecco-Telethon Institute, Venetian Institute of Molecular Medicine, Via Orus 2, 35129 Padova, Italy; Department of Biology, University of Padova, Via U. Bassi 58B, 35121 Padova, Italy.
Cell. 2013 Sep 26;155(1):160-71. doi: 10.1016/j.cell.2013.08.032. Epub 2013 Sep 19.
Respiratory chain complexes assemble into functional quaternary structures called supercomplexes (RCS) within the folds of the inner mitochondrial membrane, or cristae. Here, we investigate the relationship between respiratory function and mitochondrial ultrastructure and provide evidence that cristae shape determines the assembly and stability of RCS and hence mitochondrial respiratory efficiency. Genetic and apoptotic manipulations of cristae structure affect assembly and activity of RCS in vitro and in vivo, independently of changes to mitochondrial protein synthesis or apoptotic outer mitochondrial membrane permeabilization. We demonstrate that, accordingly, the efficiency of mitochondria-dependent cell growth depends on cristae shape. Thus, RCS assembly emerges as a link between membrane morphology and function.
呼吸链复合物在内膜褶皱或嵴内组装成称为超级复合物 (RCS) 的功能四聚体结构。在这里,我们研究了呼吸功能与线粒体超微结构之间的关系,并提供了证据表明嵴的形状决定了 RCS 的组装和稳定性,从而决定了线粒体呼吸效率。嵴结构的遗传和凋亡操作会影响 RCS 在体外和体内的组装和活性,而与线粒体蛋白合成或凋亡外膜通透性的变化无关。我们证明,相应地,依赖线粒体的细胞生长效率取决于嵴的形状。因此,RCS 组装成为膜形态和功能之间的联系。