Zanaboni G, Grimm R, Dyne K M, Rossi A, Cetta G, Iadarola P
Dipartimento di Biochimica, Università di Pavia, Italy.
J Chromatogr B Biomed Appl. 1996 Aug 9;683(1):97-107. doi: 10.1016/0378-4347(95)00583-8.
Prolidase deficiency (PD) is characterized by massive urinary excretion of imidodipeptides X-Pro and X-Hyp. We report the applicability of capillary zone electrophoresis to urinary imidodipeptide determination. The protocol is fast, simple, reliable, only small amounts of sample are required and there is minimal sample preparation. Electropherograms of urine samples from control subjects and four patients with prolidase deficiency were compared. The presence of imidodipeptides normally absent in urine was evident in patients' urine. Further analysis of urine samples enabled identification of excreted imidodipeptides and the pattern of excretion appeared to be heterogeneous for different patients. This method appears to be useful for identification of imidodipeptides in biological samples, as an efficient aid in diagnosis of PD, and as a method for providing more information about this disease.
脯氨酰二肽氨基肽酶缺乏症（PD）的特征是大量尿中排出亚氨基二肽X - Pro和X - Hyp。我们报告了毛细管区带电泳在尿亚氨基二肽测定中的适用性。该方法快速、简单、可靠，仅需少量样品且样品制备极少。比较了对照受试者和四名脯氨酰二肽氨基肽酶缺乏症患者的尿液样品电泳图。患者尿液中明显存在正常尿液中不存在的亚氨基二肽。对尿液样品的进一步分析能够鉴定出排泄的亚氨基二肽，并且不同患者的排泄模式似乎是异质性的。该方法似乎可用于鉴定生物样品中的亚氨基二肽，作为诊断PD的有效辅助手段，以及作为提供有关该疾病更多信息的方法。
