TY - JOUR
T1 - Liquid chromatographic-atmospheric pressure chemical ionization mass spectrometric analysis of glycine conjugates and urinary isovalerylglycine in isovaleric acidemia
AU - Ito, Tetsuya
AU - Kidouchi, Kiyoshi
AU - Sugiyama, Naruji
AU - Kajita, Mitsuharu
AU - Chiba, Taku
AU - Niwa, Toshimitsu
AU - Wada, Yoshiro
PY - 1995/8/18
Y1 - 1995/8/18
N2 - 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.
AB - 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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U2 - 10.1016/0378-4347(95)00174-3
DO - 10.1016/0378-4347(95)00174-3
M3 - Article
C2 - 8548022
AN - SCOPUS:0029102960
SN - 0378-4347
VL - 670
SP - 317
EP - 322
JO - Journal of Chromatography B: Biomedical Sciences and Applications
JF - Journal of Chromatography B: Biomedical Sciences and Applications
IS - 2
ER -