| Research Abstract |
In order to perform the pathophysiologic analysis in disorders of sugar and organic acid metabolism, the methods to profile the abnormal metabolites in body fluids of the patients with these disorders were investigated. Quantitative and profiling analyses of sugar, sugar alcohol and sugar acid were done using capillary gas chromatography and mass spectrometry. Using this technique, a series of unique aldonic acids were found in the urine samples of healthy newboms and diabetic human and canine. The analysis of plasma and urine using this method revealed that these aldonic acids were produced by canine after aldose loading. Capillary gas chromatographic analysis of trimethylsilyl acid fraction extracted from the urine using an ion exchange method was found to be a practical and useful method to diagnose fructose- 1, 6diphosphatase deficiency. In addition, diagnostically important sugar phosphates, fructose- 1, 6diphosphate and 3-phosphoglycerate, and glycemldehyde were found to be excreted in the urines of the patients with this disorder. Since some ketose phosphates, especially fructose-1, 6diphosphate, were degenerated to some extent during derivatization, however, the measurements of underivatized sample were studied. Although FAB-MS/MS analysis was not a sensitive method to measure these sugar phosphates, LC/MS/MS with ionspray interface was found to produce molecular ions of sugar phosphates with high intensity, which seems to enable MS/MS analysis. It is thought that the latter technique may be a sensitive and useful method to profile sugar phosphate in biological samples.
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