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The MICOS complex, a structural element of mitochondria with versatile functions

机译:Micos复合物,具有多功能功能的线粒体的结构元素

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Mitochondria perform a plethora of functions in various cells of different tissues. Their architecture differs remarkably, for instance in neurons versus steroidogenic cells. Furthermore, aberrant mitochondrial architecture results in mitochondrial dysfunction. This indicates strongly that mitochondrial architecture and function are intimately linked. Therefore, a deep knowledge about the determinants of mitochondrial architecture and their function on a molecular level is of utmost importance. In the past decades, various proteins and protein complexes essential for formation of mitochondrial architecture have been identified. Here we will review the current knowledge of the MICOS complex, one of the major structural elements of mitochondria. MICOS is a multi-subunit complex present in the inner mitochondrial membrane. Multiple interaction partners in the inner and outer mitochondrial membrane point to participation in a multitude of important processes, such as generation of mitochondrial architecture, lipid metabolism, and protein import into mitochondria. Since the MICOS complex is highly conserved in form and function throughout evolution, we will highlight the importance of MICOS for mammals. We will emphasize in particular the current knowledge of the association of MICOS with severe human diseases, including Charcot–Marie–Tooth disease type 2, Alzheimer's disease, Parkinson's disease, Frontotemporal Dementia and Amyotrophic Lateral Sclerosis.
机译:线粒体在不同组织的各种细胞中进行血清功能。它们的结构显着不同,例如在神经元与类固化细胞中。此外,异常线粒体架构导致线粒体功能障碍。这强烈表示线粒体架构和功能密切相关。因此,关于线粒体架构的决定因素及其在分子水平上的功能的深度知识至关重要。在过去的几十年中,已经确定了对形成线粒体结构的各种蛋白质和蛋白质复合物。在这里,我们将审查Micos Complex的目前的知识,线粒体的主要结构元素之一。 MICOS是内部线粒体膜中存在的多亚基复合物。内外线粒体膜中的多个相互作用伙伴指向大量重要过程,例如产生线粒体建筑,脂质代谢和蛋白质进口到线粒体中的产生。由于米多斯复合体在整个进化过程中高度保守,因此我们将突出MICOS对哺乳动物的重要性。我们将特别强调目前对MICOS协会具有严重的人类疾病的知识,包括Charcot-Marie-Tooth疾病2型,Alzheimer病,帕金森病,额颞造血症和肌营养的外侧硬化。

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