The effect of D-(+)-glucosamine on levels of free N-acetylneuraminic acid and UDP-N-acetylhexosamines in infantile sialic acid storage disease (ISSD) fibroblasts.

Abstract	An impairment in the regulation of N-acetylneuraminic acid (NANA) biosynthesis might potentially contribute to accumulation of free NANA in fibroblasts of patients with sialic acid storage disease (ISSD). By the use of a glucosamine loading test an increase in uridine-diphosphate-N-acetyl-hexosamines (UDP-HexNAc) but not in free NANA was found. NANA biosynthesis therefore appears to be under normal regulatory control in ISSD.
Authors	E Paschke, G Höfler, A Roscher
Journal	Journal of inherited metabolic disease (J Inherit Metab Dis) Vol. 10 Issue 1 Pg. 48-51 ( 1987) ISSN: 0141-8955 [Print] United States
PMID	3106716 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References	Sialic Acids Uridine Diphosphate Sugars Uridine Diphosphate N-Acetylglucosamine N-Acetylneuraminic Acid Glucosamine
Topics	Fibroblasts (analysis) Glucosamine (pharmacology) Humans Infant Male Metabolism, Inborn Errors (metabolism) N-Acetylneuraminic Acid Sialic Acids (analysis, metabolism) Uridine Diphosphate N-Acetylglucosamine (analysis) Uridine Diphosphate Sugars (analysis)

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