Kidouchi K, Sugiyama N, Morishita H, Kobayashi M, Wada Y, Nohara D
Clin Chim Acta. 1987 May 15;164(3):261-6. doi: 10.1016/0009-8981(87)90300-7.
A technique for the identification of glutarylcarnitine in urine from a patient with glutaric aciduria type 1 is described. The patient's urine sample was partially purified using an anion exchange column and analyzed by a carboxylic acid analyzer fitted with an ODS reverse-phase column. The chromatogram of the patient's urine sample revealed 3 different peaks, which corresponded respectively to those of carnitine with amino acids, acetylcarnitine and glutarylcarnitine. Following hydrolysis of the sample, the chromatogram had no peaks of acetylcarnitine and glutarylcarnitine but had remarkably amplified peaks of carnitine, acetic acid and glutaric acid. The eluent fraction of glutarylcarnitine from the non-hydrolyzed sample was hydrolyzed and analyzed again. It no longer had the glutarylcarnitine peak on the chromatogram, but had only two separate peaks of carnitine and glutaric acid. This technique simplifies the identification of glutarylcarnitine, in that it requires only removal of organic acids for preparation of samples, and does not require radioisotope or mass spectrometry.
本文描述了一种用于鉴定1型戊二酸血症患者尿液中戊二酰肉碱的技术。患者的尿液样本通过阴离子交换柱进行部分纯化，并使用配备ODS反相柱的羧酸分析仪进行分析。患者尿液样本的色谱图显示出3个不同的峰，分别对应于肉碱与氨基酸、乙酰肉碱和戊二酰肉碱的峰。样品水解后，色谱图中没有乙酰肉碱和戊二酰肉碱的峰，但肉碱、乙酸和戊二酸的峰显著放大。对未水解样品中戊二酰肉碱的洗脱级分进行水解并再次分析。色谱图上不再有戊二酰肉碱峰，只有肉碱和戊二酸两个单独的峰。该技术简化了戊二酰肉碱的鉴定，因为它只需要去除有机酸来制备样品，不需要放射性同位素或质谱分析。
