Mitochondrial β-oxidation is the most prominent pathway for fatty acid oxidation but alternative oxidative metabolism exists. Fatty acid ω-oxidation is one of these pathways and forms dicarboxylic acids as products. These dicarboxylic acids are metabolized through peroxisomal β-oxidation representing an alternative pathway, which could potentially limit the toxic effects of fatty acid accumulation. Although dicarboxylic acid metabolism is highly active in liver and kidney, its role in physiology has not been explored in depth. In this review, we summarize the biochemical mechanism of the formation and degradation of dicarboxylic acids through ω- and β-oxidation, respectively. We will discuss the role of dicarboxylic acids in different (patho)physiological states with a particular focus on the role of the intermediates and products generated through peroxisomal β-oxidation. This review is expected to increase the understanding of dicarboxylic acid metabolism and spark future research.

中文翻译：

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长链二羧酸代谢的生物化学和生理学


线粒体β-氧化是脂肪酸氧化最重要的途径，但也存在替代的氧化代谢。脂肪酸ω-氧化是这些途径之一，并形成二羧酸作为产物。这些二羧酸通过过氧化物酶体β-氧化代谢，代表另一种途径，这可能会限制脂肪酸积累的毒性作用。尽管二羧酸代谢在肝脏和肾脏中高度活跃，但其生理作用尚未深入探讨。在这篇综述中，我们分别总结了通过ω-和β-氧化形成和降解二羧酸的生化机制。我们将讨论二羧酸在不同（病理）生理状态中的作用，特别关注通过过氧化物酶体β-氧化产生的中间体和产物的作用。这篇综述预计将增加对二羧酸代谢的理解并激发未来的研究。