Isolation and identification of 2-deoxy-2,3-dehydro-N-acetylneuraminic acid from the urine of a patient with sialuria.
N-Acetylneuraminic acid preparations from the urine of a patient with sialuria contain 1--2% of 2-deoxy-2,3-dehydro-N-acetylneuraminic acid. This new human sialic acid was isolated by ion-exchange and partition chromatography. The structure has been elucidated by mass spectrometry and confirmed by comparison with the synthetic compound. The properties of this unsaturated sialic acid in the orcinol/Fe3+/HCl and the periodic acid/thiobarbituric acid tests as well as in thin-layer and gas-liquid chromatography are described. It does not react with acylneuraminate pyruvatelyase. The origin of this new human sialic acid is discussed.
D002239:Carbohydrate Metabolism, Inborn Errors / Q000652:urine*; D002648:Child; D002849:Chromatography, Gas; D002855:Chromatography, Thin Layer; D006801:Humans; D008607:Intellectual Disability / Q000652:urine; D008297:Male; D013058:Mass Spectrometry; D012794:Sialic Acids / Q000652:urine*
D012794:Sialic Acids