Document Detail

The effect of D-(+)-glucosamine on levels of free N-acetylneuraminic acid and UDP-N-acetylhexosamines in infantile sialic acid storage disease (ISSD) fibroblasts.
MedLine Citation:
PMID:  3106716     Owner:  NLM     Status:  MEDLINE    
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.
E Paschke; G Höfler; A Roscher
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of inherited metabolic disease     Volume:  10     ISSN:  0141-8955     ISO Abbreviation:  J. Inherit. Metab. Dis.     Publication Date:  1987  
Date Detail:
Created Date:  1987-06-05     Completed Date:  1987-06-05     Revised Date:  2007-03-21    
Medline Journal Info:
Nlm Unique ID:  7910918     Medline TA:  J Inherit Metab Dis     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  48-51     Citation Subset:  IM    
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MeSH Terms
Fibroblasts / analysis
Glucosamine / pharmacology*
Metabolism, Inborn Errors / metabolism*
N-Acetylneuraminic Acid
Sialic Acids / analysis,  metabolism*
Uridine Diphosphate N-Acetylglucosamine / analysis
Uridine Diphosphate Sugars / analysis*
Reg. No./Substance:
0/Sialic Acids; 0/Uridine Diphosphate Sugars; 131-48-6/N-Acetylneuraminic Acid; 3416-24-8/Glucosamine; 528-04-1/Uridine Diphosphate N-Acetylglucosamine

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