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Selected Publication:
Paschke, E; Höfler, G; Roscher, A.
The effect of D-(+)-glucosamine on levels of free N-acetylneuraminic acid and UDP-N-acetylhexosamines in infantile sialic acid storage disease (ISSD) fibroblasts.
J Inherit Metab Dis. 1987; 10(1):48-51
Doi: 10.1007/BF01799487
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- Leading authors Med Uni Graz
- Paschke Eduard
- Co-authors Med Uni Graz
- Höfler Gerald
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- 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.
- Find related publications in this database (using NLM MeSH Indexing)
- Fibroblasts - analysis
- Glucosamine - pharmacology
- Humans - pharmacology
- Infant - pharmacology
- Male - pharmacology
- Metabolism, Inborn Errors - metabolism
- N-Acetylneuraminic Acid - metabolism
- Sialic Acids - analysis
- Uridine Diphosphate N-Acetylglucosamine - analysis
- Uridine Diphosphate Sugars - analysis