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首页> 外文期刊>Current Biology: CB >Impact of Mitochondrial Fatty Acid Synthesis on?Mitochondrial Biogenesis
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Impact of Mitochondrial Fatty Acid Synthesis on?Mitochondrial Biogenesis

机译：线粒体脂肪酸合成对α线粒体生物发生的影响

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摘要

Biology students today are taught that mitochondria are ‘the powerhouse of the cell’. This gross over-simplification of their cellular role has arguably led to a paucity of knowledge concerning the many other tasks carried out by this multifunctional organelle. Mitochondrial fatty acid synthesis (mtFAS) is one such under-appreciated pathway that is crucial for mitochondrial function, although even mitochondrial experts are often surprised to learn of its existence. For many years, the only function of mtFAS was thought to be the production of lipoic acid, an important co-factor for several mitochondrial enzymes. However, recent advances have revealed a far wider role for mtFAS in mitochondrial physiology. The discovery of human patients with mutations in mtFAS enzymes has brought renewed interest in understanding the full significance of this novel mode of mitochondrial metabolic regulation. We now appreciate that mtFAS is a nutrient-sensitive pathway that provides an elegant mechanism whereby acetyl-CoA regulates its own consumption via coordination of lipoic acid synthesis and tricarboxylic acid (TCA) cycle activity, iron–sulfur (FeS) cluster biogenesis, assembly of oxidative phosphorylation complexes, and mitochondrial translation. In this minireview, we describe and build upon the important discoveries that led to our current understanding of this elegant mechanism of coordination of nutrient status and metabolism.
机译：今天的生物学学生被教导，线粒体是“细胞的强国”。这种毛细管角色的粗略简化可谓是关于该多功能细胞器进行的许多其他任务的知识。线粒体脂肪酸合成（MTFAs）是一种如此不受欢迎的途径，对于线粒体功能至关重要，但甚至线粒体专家常常惊讶地令人惊讶地吸引其存在。多年来，MTFA的唯一功能被认为是硫辛酸的生产，这是几种线粒体酶的重要共同因素。然而，最近的进展揭示了MTFA在线粒体生理学中的更广泛的作用。在MTFAS酶突变中发现人类突变患者已经为了解这种新型线粒体代谢调节模式的全部意义而引起了兴趣。现在我们现在欣赏MTFA是一种营养素敏感的途径，提供优雅的机制，由此通过协调硫辛酸合成和三羧酸（TCA）循环活性，铁 - 硫（FES）簇生物发生，组装来调节其自身的消耗。氧化磷酸化复合物和线粒体翻译。在这个MINIREVIEW中，我们描述并建立在重要的发现，导致我们目前对这种营养状况和新陈代谢协调的这种优雅机制的理解。

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