Profiling Analysis of Sugar Phosphates, Sugar Alcohols and Sugar Acids and Its Application to The Studies in Disorders of Sugar Metabolism.

为了对糖、有机酸代谢紊乱进行病理生理分析，对糖、有机酸代谢紊乱患者体液中异常代谢物的分析方法进行了探讨。采用毛细管气相色谱-质谱法对糖、糖醇、糖酸进行了定量和图谱分析。利用这项技术，在健康新生儿和糖尿病人和狗的尿样中发现了一系列独特的醛酸。用该方法对血浆和尿液的分析表明，这些醛酸是犬在醛糖负荷后产生的。用离子交换法从尿液中提取的三甲基硅酸组分的毛细管气相色谱分析是诊断果糖-1，6-二磷酸酶缺乏症的一种实用而有用的方法。此外，诊断上重要的糖磷酸盐、1，6-二磷酸果糖和3-磷酸甘油酸，以及乙二醛被发现排出在患者的尿液中。但是，由于一些酮糖磷酸，特别是1，6-二磷酸果糖在衍生化过程中会发生不同程度的降解，因此对未衍生化样品的测定进行了研究。虽然FAB-MS/MS分析不是一种灵敏的方法来测定这些糖磷酸盐，但有离子喷雾界面的LC/MS/MS被发现能产生高强度的糖磷酸盐分子离子，这似乎使MS/MS分析成为可能。认为后一种技术可能是一种灵敏而有用的方法来分析生物样品中的糖磷酸盐。

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.