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Journal of Inherited Metabolic Disease

, Volume 9, Issue 1, pp 31–38 | Cite as

High-performance liquid chromatographic assay of argininosuccinate: its application in argininosuccinic aciduria and in normal man

  • M. Portolés
  • V. Rubio
Article

Abstract

Argininosuccinate (ASA) is determined in a few minutes with little manipulation by reversed phase high-performance liquid chromatography (HPLC) usingO-phthaldialdehyde. The two cyclic anhydrides of ASA are not formed during analysis but, if present, can be determined simultaneously. As little as 1 nl urine from a patient with argininosuccinic aciduria was sufficient for analysis; the ASA/creatinine ratio was 50.8 mmol g−1 and daily excretion was 5–7g ASA. We found small amounts of the two anhydrides in the patient's urine and we give factors to estimate, from their peaks, the corresponding amount of ASA. Urine from normal children showed a small acid-labile (at 100°C) peak at the ASA position, which we tentatively assign to genuine ASA. From this peak <2mg ASAday−1 were excreted in our controls. Procedures for collection and storage of samples and the potential of this method for heterozygote detection are discussed.

Keywords

Public Health Internal Medicine Liquid Chromatography Anhydride Metabolic Disease 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© SSIEM and MTP Press Limited 1986

Authors and Affiliations

  • M. Portolés
    • 1
  • V. Rubio
    • 1
  1. 1.Instituto de Investigaciones Citológicas de la Caja de Ahorros de ValenciaValenciaSpain

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