Oxidative phosphorylation (OXPHOS) occurs through and across the inner mitochondrial membrane (IMM). Mitochondrial membranes contain a distinct lipid composition, aided by lipid biosynthetic machinery localized in the IMM and class-specific lipid transporters that limit lipid traffic in and out of mitochondria. This unique lipid composition appears to be essential for functions of mitochondria, particularly OXPHOS, by its effects on direct lipid-to-protein interactions, membrane properties, and cristae ultrastructure. This review highlights the biological significance of mitochondrial lipids, with a particular spotlight on the role of lipids in mitochondrial bioenergetics. We describe pathways for the biosynthesis of mitochondrial lipids and provide evidence for their roles in physiology, their implications in human disease, and the mechanisms by which they regulate mitochondrial bioenergetics.

氧化磷酸化 (OXPHOS) 发生在线粒体内膜 (IMM) 上。线粒体膜含有独特的脂质成分，在 IMM 中定位的脂质生物合成机制和限制脂质进出线粒体的特定类脂质转运蛋白的帮助下。这种独特的脂质成分似乎对线粒体的功能至关重要，特别是 OXPHOS，因为它对脂质与蛋白质的直接相互作用、膜特性和嵴超微结构有影响。这篇综述强调了线粒体脂质的生物学意义，特别关注脂质在线粒体生物能学中的作用。我们描述了线粒体脂质生物合成的途径，并为它们在生理学中的作用、它们对人类疾病的影响以及它们调节线粒体生物能的机制提供了证据。