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Simple Method for the Quantitative Analysis of Dihydropyrimidines and N-Carbamyl-ß-Amino Acids in Urine

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Part of the Advances in Experimental Medicine and Biology book series (AEMB,volume 309B)

Abstract

In man pyrimidines are degraded in four steps catalysed by dihydropyrimidine dehydrogenase (DHPD, EC 1. 3. 1. 2.), dihydropyrimidinase (DHP, EC 3. 5. 2. 2.), ureidopropionase (UP, EC 3. 5. 1. 6.) and a transaminase (Fig. 1). As yet only deficiencies of the first and the last enzymes have been described. Deficiency of R-ß-aminoisobutyrate aminotransferase (EC 2. 6. 1. 22.) is easily detected by classical amino acid analysis 12,DHPD-deficiency by TLC or HPLC of pyrimidines in urine3. A method is presented for the detection of the two other defects.

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References

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© 1991 Plenum Press, New York

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van Gennip, A.H., Busch, S., Scholten, E.G., Stroomer, L.E., Abeling, N.G. (1991). Simple Method for the Quantitative Analysis of Dihydropyrimidines and N-Carbamyl-ß-Amino Acids in Urine. In: Harkness, R.A., Elion, G.B., Zöllner, N. (eds) Purine and Pyrimidine Metabolism in Man VII. Advances in Experimental Medicine and Biology, vol 309B. Springer, New York, NY. https://doi.org/10.1007/978-1-4615-7703-4_4

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  • DOI: https://doi.org/10.1007/978-1-4615-7703-4_4

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4615-7705-8

  • Online ISBN: 978-1-4615-7703-4

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