Kamerling J P, Strecker G, Farriaux J P, Dorland L, Haverkamp J, Vliegenthart J F
Biochim Biophys Acta. 1979 Mar 22;583(3):403-8. doi: 10.1016/0304-4165(79)90465-3.
A new metabolite, namely 2-acetamidoglucal, has been found in the urine of a patient with sialuria in addition to the metabolites N-acetylneuraminic acid, N-acetylmannosamine, N-acetylglucosamine and 2-deoxy-2,3-dehydro-N-acetylneuraminic acid reported earlier. the structure has been identified by mass spectrometry and 360 MHz proton nuclear magnetic resonance spectroscopy and verified by synthesis. All accumulated compounds fit into the metabolic pathway for the biosynthesis of CMP-N-acetylneuraminic acid. Sialuria is discussed in terms of a failure of regulation of UDP-N-acetylglucosamine 2-epimerase.
除了先前报道的代谢产物N-乙酰神经氨酸、N-乙酰甘露糖胺、N-乙酰葡糖胺和2-脱氧-2,3-脱氢-N-乙酰神经氨酸外，在一名患有多涎症的患者尿液中发现了一种新的代谢产物，即2-乙酰氨基葡糖醛。其结构已通过质谱和360兆赫质子核磁共振光谱鉴定，并通过合成进行了验证。所有积累的化合物都符合CMP-N-乙酰神经氨酸生物合成的代谢途径。从UDP-N-乙酰葡糖胺2-差向异构酶调节失败的角度讨论了多涎症。
