Department of Pediatrics, Shimane University School of Medicine, 89-1 En-ya-cho, Izumo, Shimane 693-8501, Japan.
J Chromatogr B Analyt Technol Biomed Life Sci. 2010 Nov 15;878(30):3113-8. doi: 10.1016/j.jchromb.2010.09.015. Epub 2010 Sep 29.
A simple and rapid method for quantitative analysis of amino acids, including valine (Val), leucine (Leu), isoleucine (Ile), methionine (Met) and phenylalanine (Phe), in whole blood has been developed using GC/MS. In this method, whole blood was collected using a filter paper technique, and a 1/8 in. blood spot punch was used for sample preparation. Amino acids were extracted from the sample, and the extracts were purified using cation-exchange resins. The isotope dilution method using ²H₈-Val, ²H₃-Leu, ²H₃-Met and ²H₅-Phe as internal standards was applied. Following propyl chloroformate derivatization, the derivatives were analyzed using fast-GC/MS. The extraction recoveries using these techniques ranged from 69.8% to 87.9%, and analysis time for each sample was approximately 26 min. Calibration curves at concentrations from 0.0 to 1666.7 μmol/l for Val, Leu, Ile and Phe and from 0.0 to 333.3 μmol/l for Met showed good linearity with regression coefficients=1. The method detection limits for Val, Leu, Ile, Met and Phe were 24.2, 16.7, 8.7, 1.5 and 12.9 μmol/l, respectively. This method was applied to blood spot samples obtained from patients with phenylketonuria (PKU), maple syrup urine disease (MSUD), hypermethionine and neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), and the analysis results showed that the concentrations of amino acids that characterize these diseases were increased. These results indicate that this method provides a simple and rapid procedure for precise determination of amino acids in whole blood.
已开发出一种使用 GC/MS 对全血中的氨基酸(包括缬氨酸(Val)、亮氨酸(Leu)、异亮氨酸(Ile)、蛋氨酸(Met)和苯丙氨酸(Phe))进行定量分析的简单快速方法。在该方法中,使用滤纸技术采集全血,并用 1/8 英寸血斑打孔器进行样品制备。从样品中提取氨基酸,并用阳离子交换树脂对提取物进行纯化。应用同位素稀释法,使用 ²H₈-Val、²H₃-Leu、²H₃-Met 和 ²H₅-Phe 作为内标。经过丙酰氯甲酸酯衍生化后,使用快速 GC/MS 分析衍生物。这些技术的提取回收率在 69.8%至 87.9%之间,每个样品的分析时间约为 26 分钟。在 0.0 至 1666.7 μmol/l 浓度范围内,Val、Leu、Ile 和 Phe 的校准曲线以及在 0.0 至 333.3 μmol/l 浓度范围内的 Met 的校准曲线均呈良好线性,回归系数=1。Val、Leu、Ile、Met 和 Phe 的方法检测限分别为 24.2、16.7、8.7、1.5 和 12.9 μmol/l。该方法应用于从苯丙酮尿症(PKU)、枫糖尿症(MSUD)、高蛋氨酸血症和 citrin 缺乏引起的新生儿肝内胆汁淤积症(NICCD)患者的血斑样本中,分析结果表明,这些疾病特征性的氨基酸浓度升高。这些结果表明,该方法为全血中氨基酸的精确测定提供了一种简单快速的程序。