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
; 10(1): 48-51, 1987.
Article
en En
| MEDLINE
| ID: mdl-3106716
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.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Ácidos Siálicos
/
Azúcares de Uridina Difosfato
/
Glucosamina
/
Errores Innatos del Metabolismo
Límite:
Humans
/
Infant
/
Male
Idioma:
En
Revista:
J Inherit Metab Dis
Año:
1987
Tipo del documento:
Article