The effect ofd-(+)-glucosamine on levels of freeN-acetylneuraminic acid and UDP-N-acetylhexosamines in infantile sialic acid storage disease (ISSD) fibroblasts |
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| Authors: | E. Paschke G. Höfler A. Roscher |
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| Affiliation: | (1) Department of Pediatrics, University of Graz, Auenbruggerplatz, A-8036 Graz, Austria |
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| Abstract: | An impairment in the regulation ofN-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. |
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