Laboratory of Mitochondrial Biogenesis, International Institute of Molecular and Cell Biology, Warsaw 02-109, Poland.
Department of Microbiology, Monash University, Melbourne, Victoria 3800, Australia; Victorian Bioinformatics Consortium, Monash University, Melbourne, Victoria 3800, Australia.
FEBS Lett. 2014 Aug 1;588(15):2484-95. doi: 10.1016/j.febslet.2014.05.028. Epub 2014 May 24.
Mitochondria are involved in many essential cellular activities. These broad functions explicate the need for the well-orchestrated biogenesis of mitochondrial proteins to avoid death and pathological consequences, both in unicellular and more complex organisms. Yeast as a model organism has been pivotal in identifying components and mechanisms that drive the transport and sorting of nuclear-encoded mitochondrial proteins. The machinery components that are involved in the import of mitochondrial proteins are generally evolutionarily conserved within the eukaryotic kingdom. However, topological and functional differences have been observed. We review the similarities and differences in mitochondrial translocases from yeast to human. Additionally, we provide a systematic overview of the contribution of mitochondrial import machineries to human pathologies, including cancer, mitochondrial diseases, and neurodegeneration.
线粒体参与许多重要的细胞活动。这些广泛的功能解释了需要协调线粒体蛋白质的生物发生，以避免死亡和病理后果，无论是在单细胞生物还是更复杂的生物中。酵母作为一种模式生物，对于识别驱动核编码线粒体蛋白质的运输和分拣的成分和机制至关重要。参与线粒体蛋白质输入的机器成分在真核生物王国中通常是进化保守的。然而，已经观察到拓扑和功能上的差异。我们回顾了从酵母到人线粒体转位酶的相似性和差异。此外，我们还系统地概述了线粒体输入机制对人类疾病的贡献，包括癌症、线粒体疾病和神经退行性变。
