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

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)317-322
Number of pages6
JournalJournal of Chromatography B: Biomedical Sciences and Applications
Volume670
Issue number2
DOIs
Publication statusPublished - 18-08-1995
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Chemistry(all)

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