Use of capillary zone electrophoresis for analysis of imidodipeptides in urine of prolidase-deficient patients.

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