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Dominant optic atrophy, OPA1, and mitochondrial quality control: understanding mitochondrial network dynamics

机译：优势视神经萎缩，OPA1和线粒体质量控制：了解线粒体网络动力学

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Mitochondrial quality control is fundamental to all neurodegenerative diseases, including the most prominent ones, Alzheimer’s Disease and Parkinsonism. It is accomplished by mitochondrial network dynamics – continuous fission and fusion of mitochondria. Mitochondrial fission is facilitated by DRP1, while MFN1 and MFN2 on the mitochondrial outer membrane and OPA1 on the mitochondrial inner membrane are essential for mitochondrial fusion. Mitochondrial network dynamics are regulated in highly sophisticated ways by various different posttranslational modifications, such as phosphorylation, ubiquitination, and proteolytic processing of their key-proteins. By this, mitochondria process a wide range of different intracellular and extracellular parameters in order to adapt mitochondrial function to actual energetic and metabolic demands of the host cell, attenuate mitochondrial damage, recycle dysfunctional mitochondria via the mitochondrial autophagy pathway, or arrange for the recycling of the complete host cell by apoptosis. Most of the genes coding for proteins involved in this process have been associated with neurodegenerative diseases. Mutations in one of these genes are associated with a neurodegenerative disease that originally was described to affect retinal ganglion cells only. Since more and more evidence shows that other cell types are affected as well, we would like to discuss the pathology of dominant optic atrophy, which is caused by heterozygous sequence variants in OPA1, in the light of the current view on OPA1 protein function in mitochondrial quality control, in particular on its function in mitochondrial fusion and cytochrome C release. We think OPA1 is a good example to understand the molecular basis for mitochondrial network dynamics.
机译：线粒体质量控制是所有神经退行性疾病（包括最突出的疾病），阿尔茨海默氏病和帕金森病的基础。它是通过线粒体网络动力学-线粒体的连续裂变和融合来实现的。 DRP1促进线粒体裂变，而线粒体外膜上的MFN1和MFN2以及线粒体内膜上的OPA1对于线粒体融合至关重要。线粒体网络动力学通过各种不同的翻译后修饰（例如其关键蛋白的磷酸化，泛素化和蛋白水解加工）以高度复杂的方式进行调控。通过这种方式，线粒体可以处理各种不同的细胞内和细胞外参数，以使线粒体功能适应宿主细胞的实际能量和代谢需求，减轻线粒体损伤，通过线粒体自噬途径回收功能障碍的线粒体或安排回收利用完整的宿主细胞通过凋亡。编码参与该过程的蛋白质的大多数基因都与神经退行性疾病有关。这些基因之一的突变与神经退行性疾病有关，该疾病最初被描述为仅影响视网膜神经节细胞。由于越来越多的证据表明其他细胞类型也受到影响，因此我们希望根据线粒体中OPA1蛋白功能的当前观点，讨论由视神经乳头蛋白1（OPA1）杂合序列变异引起的显性视神经萎缩的病理学质量控制，尤其是其在线粒体融合和细胞色素C释放中的功能。我们认为OPA1是了解线粒体网络动力学分子基础的一个很好的例子。

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