Department of Genetics and Genomic Sciences, Icahn Institute for Data Science and Genomic Technology, Icahn School of Medicine at Mount Sinai, 1425 Madison Avenue, Box 1498, New York, NY 10029, USA.
Laboratory Genetic Metabolic Diseases, Amsterdam UMC, University of Amsterdam, Department of Clinical Chemistry, Amsterdam Gastroenterology & Metabolism, Meibergdreef 9, 1105 AZ Amsterdam, the Netherlands.
Biochim Biophys Acta Mol Basis Dis. 2020 May 1;1866(5):165720. doi: 10.1016/j.bbadis.2020.165720. Epub 2020 Feb 10.
Carnitine plays an essential role in mitochondrial fatty acid β-oxidation as a part of a cycle that transfers long-chain fatty acids across the mitochondrial membrane and involves two carnitine palmitoyltransferases (CPT1 and CPT2). Two distinct carnitine acyltransferases, carnitine octanoyltransferase (COT) and carnitine acetyltransferase (CAT), are peroxisomal enzymes, which indicates that carnitine is not only important for mitochondrial, but also for peroxisomal metabolism. It has been demonstrated that after peroxisomal metabolism, specific intermediates can be exported as acylcarnitines for subsequent and final mitochondrial metabolism. There is also evidence that peroxisomes are able to degrade fatty acids that are typically handled by mitochondria possibly after transport as acylcarnitines. Here we review the biochemistry and physiological functions of metabolite exchange between peroxisomes and mitochondria with a special focus on acylcarnitines.
肉碱在作为长链脂肪酸穿过线粒体膜的循环的一部分的线粒体脂肪酸β-氧化中起着重要作用，该循环涉及两种肉碱棕榈酰转移酶（CPT1 和 CPT2）。两种不同的肉碱酰基转移酶，肉碱辛酰基转移酶（COT）和肉碱乙酰基转移酶（CAT），是过氧化物酶体酶，这表明肉碱不仅对线粒体，而且对过氧化物酶体代谢都很重要。已经证明，过氧化物体代谢后，特定的中间产物可以作为酰基辅酶 A 被输出，以便随后进行最终的线粒体代谢。还有证据表明，过氧化物体可能能够降解通常由线粒体处理的脂肪酸，可能是在作为酰基辅酶 A 运输之后。在这里，我们综述了过氧化物体和线粒体之间代谢物交换的生物化学和生理学功能，特别关注酰基辅酶 A。
