Hinderlich Stephan, Weidemann Wenke, Yardeni Tal, Horstkorte Rüdiger, Huizing Marjan
Department of Life Sciences and Technology, Beuth Hochschule für Technik Berlin, University of Applied Sciences, Berlin, Germany,
Top Curr Chem. 2015;366:97-137. doi: 10.1007/128_2013_464.
UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase is the key enzyme of sialic acid biosynthesis in vertebrates. It catalyzes the first two steps of the cytosolic formation of CMP-N-acetylneuraminic acid from UDP-N-acetylglucosamine. In this review we give an overview of structure, biochemistry, and genetics of the bifunctional enzyme and its complex regulation. Furthermore, we will focus on diseases related to UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase.
UDP-N-乙酰葡糖胺2-差向异构酶/N-乙酰甘露糖胺激酶是脊椎动物中唾液酸生物合成的关键酶。它催化从UDP-N-乙酰葡糖胺胞质形成CMP-N-乙酰神经氨酸的前两步反应。在本综述中,我们概述了这种双功能酶的结构、生物化学和遗传学及其复杂的调控机制。此外,我们将重点关注与UDP-N-乙酰葡糖胺2-差向异构酶/N-乙酰甘露糖胺激酶相关的疾病。