Simultaneous LC-MS/MS determination of phenylbutyrate, phenylacetate benzoate and their corresponding metabolites phenylacetylglutamine and hippurate in blood and urine
| Autor: | Thomas Meissner, Diran Herebian, Ertan Mayatepek, Maurice D. Laryea |
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| Rok vydání: | 2010 |
| Předmět: |
Spectrometry
Mass Electrospray Ionization Glutamine Phenylbutyrate Benzoates chemistry.chemical_compound Limit of Detection Predictive Value of Tests Tandem Mass Spectrometry Genetics medicine Humans Hyperammonemia Urea Cycle Disorders Inborn Genetics (clinical) Biotransformation Phenylacetates Chromatography Reverse-Phase Chromatography Hippurates Hippuric acid Reproducibility of Results Sodium phenylbutyrate Reference Standards medicine.disease Phenylbutyrates Phenylacetylglutamine chemistry Urea cycle Calibration Urea Sodium benzoate Linear Models Biomarkers medicine.drug |
| Zdroj: | Journal of inherited metabolic disease. 33 |
| ISSN: | 1573-2665 |
| Popis: | Inborn errors of urea metabolism result in hyperammonemia. Treatment of urea cycle disorders can effectively lower plasma ammonium levels and results in survival in the majority of patients. Available medications for treating urea cycle disorders include sodium benzoate (BA), sodium phenylacetate (PAA), and sodium phenylbutyrate (PBA) and are given to provide alternate routes for disposition of waste nitrogen excretion. In this study, we develop and validate a liquid chromatography tandem mass spectrometry (LC-MS/MS) method for simultaneous determination of benzoic acid, phenylacetic acid, phenylbutyric acid, phenylacetylglutamine, and hippuric acid in plasma and urine from children with inborn errors of urea synthesis. Plasma extracts and diluted urine samples were injected on a reverse-phase column and identified and quantified by selected reaction monitoring (SRM) in negative ion mode. Deuterated analogues served as internal standards. Analysis time was 7 min. Assay precision, accuracy, and linearity and sample stability were determined using enriched samples. Quantification limits of the method were 100 ng/ml (0.3-0.8 μmol/L) for all analytes, and recoveries were >90%. Inter- and intraday relative standard deviations were |
| Databáze: | OpenAIRE |
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