Azetidine-2-carboxylic acid contaminated dietary proline as a cause of urinary excretion of 4-amino-2-(S-cysteinyl)butyric acid in patients on oral treatment with a synthetic diet
| Autor: | Marinus Duran, Johannes F.G. Vliegenthart, H.J. Bremer, Sybe K. Wadman, Johannis P. Kamerling, H. Przyrembel, P.K. De Bree |
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| Rok vydání: | 1983 |
| Předmět: |
Male
Proline Food Contamination Urine Butyric acid Excretion chemistry.chemical_compound Maple Syrup Urine Disease medicine Humans Biotransformation Food Formulated chemistry.chemical_classification Chromatography Maple syrup urine disease Azetines Infant Dipeptides General Chemistry medicine.disease Amino acid chemistry Biochemistry Female Chromatography Thin Layer Azetidine-2-carboxylic acid Azetidinecarboxylic Acid Cysteine |
| Zdroj: | Journal of Chromatography B: Biomedical Sciences and Applications. 277:41-51 |
| ISSN: | 0378-4347 |
| Popis: | Three children with branched-chain ketoaciduria (maple syrup urine disease) were found to excrete an abnormal amino acid when they were on an artificial diet. This substance was identified as 4-amino-2-(S-cysteinyl)butyric acid with the use of column liquid chromatography, gas chromatography--mass spectrometry of various derivatives, and 360 MHz 1H-NMR spectroscopy. The same compound was detected in urine samples from subjects undergoing an oral loading test with L-proline. The chromatographic analysis of commercial proline from two sources indicated that one of the batches was contaminated (less than 1%) with L-azetidine-2-carboxylic acid (the homologue of proline with a four-membered ring). The latter compound is probably metabolized by the human via ring-opening and addition of a cysteine moiety. It is highly probable that the artificial diet given to the patients contained the impure proline and that the L-azetidine-2-carboxylic acid in the proline gave rise to the excretion of the 4-amino-2-(S-cysteinyl)butyric acid. |
| Databáze: | OpenAIRE |
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