D-(+)-氨基葡萄糖对婴儿唾液酸贮积病（ISSD）成纤维细胞中游离N-乙酰神经氨酸和UDP-N-乙酰己糖胺水平的影响。
The effect of D-(+)-glucosamine on levels of free N-acetylneuraminic acid and UDP-N-acetylhexosamines in infantile sialic acid storage disease (ISSD) fibroblasts.
作者信息
Paschke E, Höfler G, Roscher A
出版信息
J Inherit Metab Dis. 1987;10(1):48-51. doi: 10.1007/BF01799487.
PMID: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.
摘要
N-乙酰神经氨酸（NANA）生物合成调节的损害可能会导致唾液酸贮积病（ISSD）患者成纤维细胞中游离NANA的积累。通过使用氨基葡萄糖负荷试验，发现尿苷二磷酸-N-乙酰己糖胺（UDP-HexNAc）增加，但游离NANA未增加。因此，NANA生物合成在ISSD中似乎处于正常调节控制之下。
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