2-ACETAMIDOGLUCAL, A NEW METABOLITE ISOLATED FROM THE URINE OF A PATIENT WITH SIALURIA

被引:21
作者
KAMERLING, JP
STRECKER, G
FARRIAUX, JP
DORLAND, L
HAVERKAMP, J
VLIEGENTHART, JFG
机构
[1] CTR HOSP REG LILLE,SERV GENET & MALADIES HEREDITAIRES METAB ENFANT,LILLE,FRANCE
[2] UNIV LILLE 1,CHIM BIOL LAB,F-59650 VILLENEUVE DASCQ,FRANCE
关键词
(Human urine); 2-Acetamidoglucal; Inborn error; Sialuria;
D O I
10.1016/0304-4165(79)90465-3
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
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 N-deoxy-2,3-dehydro-Nacetylneuraminic 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-acetyl-glucosamine 2-epimerase. © 1979.
引用
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页码:403 / 408
页数:6
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