PMID- 3106716
OWN - NLM
STAT- MEDLINE
DCOM- 19870605
LR  - 20190828
IS  - 0141-8955 (Print)
IS  - 0141-8955 (Linking)
VI  - 10
IP  - 1
DP  - 1987
TI  - The effect of D-(+)-glucosamine on levels of free N-acetylneuraminic acid and 
      UDP-N-acetylhexosamines in infantile sialic acid storage disease (ISSD) 
      fibroblasts.
PG  - 48-51
AB  - 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.
FAU - Paschke, E
AU  - Paschke E
FAU - Hofler, G
AU  - Hofler G
FAU - Roscher, A
AU  - Roscher A
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Inherit Metab Dis
JT  - Journal of inherited metabolic disease
JID - 7910918
RN  - 0 (Sialic Acids)
RN  - 0 (Uridine Diphosphate Sugars)
RN  - 528-04-1 (Uridine Diphosphate N-Acetylglucosamine)
RN  - GZP2782OP0 (N-Acetylneuraminic Acid)
RN  - N08U5BOQ1K (Glucosamine)
SB  - IM
MH  - Fibroblasts/analysis
MH  - Glucosamine/*pharmacology
MH  - Humans
MH  - Infant
MH  - Male
MH  - Metabolism, Inborn Errors/*metabolism
MH  - N-Acetylneuraminic Acid
MH  - Sialic Acids/analysis/*metabolism
MH  - Uridine Diphosphate N-Acetylglucosamine/analysis
MH  - Uridine Diphosphate Sugars/*analysis
EDAT- 1987/01/01 00:00
MHDA- 1987/01/01 00:01
CRDT- 1987/01/01 00:00
PHST- 1987/01/01 00:00 [pubmed]
PHST- 1987/01/01 00:01 [medline]
PHST- 1987/01/01 00:00 [entrez]
AID - 10.1007/BF01799487 [doi]
PST - ppublish
SO  - J Inherit Metab Dis. 1987;10(1):48-51. doi: 10.1007/BF01799487.