Contrary to their well-known functions in nutrient breakdown, mitochondria are also important biosynthetic hubs and express an evolutionarily conserved mitochondrial fatty acid synthesis (mtFAS) pathway. mtFAS builds lipoic acid and longer saturated fatty acids, but its exact products, their ultimate destination in cells, and the cellular significance of the pathway are all active research questions. Moreover, why mitochondria need mtFAS despite their well-defined ability to import fatty acids is still unclear. The identification of patients with inborn errors of metabolism in mtFAS genes has sparked fresh research interest in the pathway. New mammalian models have provided insights into how mtFAS coordinates many aspects of oxidative mitochondrial metabolism and raise questions about its role in diseases such as obesity, diabetes, and heart failure. In this review, we discuss the products of mtFAS, their function, and the consequences of mtFAS impairment across models and in metabolic disease.

中文翻译：

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线粒体脂肪酸合成是哺乳动物氧化代谢的新兴中央调节剂

与众所周知的营养分解功能相反，线粒体也是重要的生物合成中心，并表达进化上保守的线粒体脂肪酸合成（mtFAS）途径。mtFAS 产生硫辛酸和较长的饱和脂肪酸，但其确切产物、它们在细胞中的最终目的地以及该途径的细胞意义都是活跃的研究问题。此外，尽管线粒体具有明确的输入脂肪酸的能力，但为什么它们需要 mtFAS 仍不清楚。mtFAS 基因代谢先天性缺陷患者的鉴定引发了对该通路的新研究兴趣。新的哺乳动物模型为 mtFAS 如何协调氧化线粒体代谢的许多方面提供了见解，并提出了有关其在肥胖、糖尿病和心力衰竭等疾病中的作用的问题。在这篇综述中，我们讨论了 mtFAS 的产物、它们的功能，以及跨模型和代谢疾病中 mtFAS 损伤的后果。