Liquid chromatographic-atmospheric pressure chemical ionization mass spectrometric analysis of glycine conjugates and urinary isovalerylglycine in isovaleric acidemia

Tetsuya Ito, Kiyoshi Kidouchi, Naruji Sugiyama, Mitsuharu Kajita, Taku Chiba, Toshimitsu Niwa, Yoshiro Wada

研究成果: Article査読

10 被引用数 (Scopus)

抄録

n-Acetylglycine, n-propionylglycine, n-butyrylglycine, isobutyrylglycine, n-valerylglycine, isovalerylglycine, heptanoylglycine, phenylacetylglycine and isovalerylglucuronide were identified based on their liquid chromatographic-atmospheric pressure chemical ionization mass spectra (LC-APCI-MS). We were able to detect the presence of urinary isovalerylglycine in two cases of isovaleric acidemia using LC-APCI-MS. Membrane-filtered urine samples were injected into the LC-APCI-MS system in the negative-ion mode without any further pretreatment, and large amounts of isovalerylglycine were detected as the [M - H]- ion. The urinary excretion of isovalerylglycine appeared to increase after l-carnitine therapy. This analytical method is quick and easy and it may be a useful tool in understanding dysfunctional conditions in isovaleric acidemia.

本文言語English
ページ(範囲)317-322
ページ数6
ジャーナルJournal of Chromatography B: Biomedical Sciences and Applications
670
2
DOI
出版ステータスPublished - 18-08-1995
外部発表はい

All Science Journal Classification (ASJC) codes

  • 化学 (全般)

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