Kong Gyeyeong, Lee Hyunji, Tran Quangdon, Kim Chaeyeong, Gong Nayoung, Park Jisoo, Kwon So Hee, Kim Seon-Hwan, Park Jongsun
Department of Pharmacology, College of Medicine, Chungnam National University, Daejeon, South Korea.
Department of Medical Science, Metabolic Syndrome and Cell Signaling Laboratory, Institute for Cancer Research, College of Medicine, Chungnam National University, Daejeon, South Korea.
Front Mol Biosci. 2020 Jul 14;7:153. doi: 10.3389/fmolb.2020.00153. eCollection 2020.
Branched chain fatty acids perform very important functions in human diet and drug metabolism. they cannot be metabolized in mitochondria and are instead processed and degraded in peroxisomes due to the presence of methyl groups on the carbon chains. Oxidative degradation pathways for lipids include α- and β-oxidation and several pathways. In all metabolic pathways, α-methyl acyl-CoA racemase (AMACR) plays an essential role by regulating the metabolism of lipids and drugs. AMACR regulates β-oxidation of branched chain lipids in peroxisomes and mitochondria and promotes chiral reversal of 2-methyl acids. AMACR defects cause sensory-motor neuronal and liver abnormalities in humans. These phenotypes are inherited and are caused by mutations in AMACR. In addition, AMACR has been found to be overexpressed in prostate cancer. In addition, the protein levels of AMACR have increased significantly in many types of cancer. Therefore, AMACR may be an important marker in tumors. In this review, a comprehensive overview of AMACR studies in human disease will be described.
支链脂肪酸在人类饮食和药物代谢中发挥着非常重要的作用。由于碳链上存在甲基，它们无法在线粒体中代谢，而是在过氧化物酶体中进行加工和降解。脂质的氧化降解途径包括α-氧化和β-氧化以及其他几种途径。在所有代谢途径中，α-甲基酰基辅酶A消旋酶（AMACR）通过调节脂质和药物的代谢发挥着至关重要的作用。AMACR调节过氧化物酶体和线粒体中支链脂质的β-氧化，并促进2-甲基酸的手性反转。AMACR缺陷会导致人类感觉运动神经元和肝脏异常。这些表型是遗传性的，由AMACR突变引起。此外，已发现AMACR在前列腺癌中过表达。此外，AMACR的蛋白质水平在多种癌症中显著升高。因此，AMACR可能是肿瘤中的一个重要标志物。在本综述中，将对人类疾病中AMACR的研究进行全面概述。
