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Liquid chromatographic-atmospheric pressure chemical ionization mass spectrometric analysis of glycine conjugates and urinary isovalerylglycine in isovaleric acidemia |
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| Authors: | Tetsuya Ito Kiyoshi Kidouchi Naruji Sugiyama Mitsuharu Kajita Taku Chiba Toshimitsu Niwa Yoshiro Wada |
| Affiliation: | a Department of Pediatrics, Nagoya City University Medical School, Aza Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467, Japan b Department of Pediatrics, Nagoya City Higashi General Hospital, Nagoya, Japan c Department of Pediatrics, Nagoya University Branch Hospital, Nagoya, Japan d Department of Hygienic Chemistry, Faculty of Pharmaceutical Sciences, Nagoya City University, Nagoya, Japan e Department of Clinical Laboratory, Nagoya University Branch Hospital, Nagoya, Japan |
| 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. |
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