The Identification and the Excretion Pattern of Isovaleryl Glucuronide in the Urine of Patients with Isovaleric Acidemia
作者:
DAVID HINE,
KAY TANAKA,
期刊:
Pediatric Research
(OVID Available online 1984)
卷期:
Volume 18,
issue 6
页码: 508-511
ISSN:0031-3998
年代: 1984
出版商: OVID
数据来源: OVID
摘要:
We identified isovaleryl glucuronide in the urine of patients with isovaleric acidemia by using gas chromatography-mass spectrometry (GC-MS) and by identifying the products of enzymatic hydrolysis. Conjugation of isovaleryl-CoA with glycine, by the action of glycine-N-acylase, is the main detoxification mechanism in isovaleric acidemia. The identification of isovaleryl glucuronide demonstrates a hitherto unknown, additional detoxification mechanism in patients with isovaleric acidemia. Quantitative analysis of 72 urine specimens from four patients with isovaleric acidemia shows that isovaleryl glucuronide is more likely to be excreted when the amount of urinary 3-hydroxyisovaleric acid excretion is high. This suggests that detoxification via glucuronide conjugation plays an important role when the glycine conjugation system is saturated.
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