Separation of free amino acids in human plasma by capillary electrophoresis with laser induced fluorescence: Potential for emergency diagnosis of inborn errors of metabolism

Olivier Boulat, David G. McLaren, Edgar A. Arriaga, David D Y Chen

Research output: Contribution to journalArticlepeer-review

64 Scopus citations

Abstract

Free amino acids (AAs) in human plasma are derivatized with 3-(4-carboxybenzoyl)quinoline-2-carboxaldehyde (CBQCA) and analyzed by capillary electrophoresis (CE) with laser induced fluorescence (LIF) detection. The labeling procedure is significantly improved over results reported previously. Derivatization can be completed in 40 min, with concentrations as low as 4×10-8 M successfully labeled in favourable cases. Twenty-nine AAs (including 2 internal standards) are identified and can be reproducibly separated in 70 min. Migration time RSD values for 23 of these AAs were calculated and found in the range from 0.5 to 4%. The rapid derivatization procedure and the resolution obtained in the separation are sufficient for a semi-quantitative, emergency diagnosis of several inborn errors of metabolism (IEM). Amino acid profiles for both normal donor plasma samples and plasma samples of patients suffering from phenylketonuria, tyrosinemia, maple syrup urinary disease, hyperornithinemia, and citrullinemia are studied.

Original languageEnglish (US)
Pages (from-to)217-228
Number of pages12
JournalJournal of Chromatography B: Biomedical Sciences and Applications
Volume754
Issue number1
DOIs
StatePublished - Apr 15 2001

Bibliographical note

Funding Information:
This work is supported by the Centre Hospitalier Universitaire Vaudois and the Laboratoire Central de Chimie Clinique of the Centre Hospitalier Universitaire Vaudois (Head: Prof. C. Bachmann) and the Natural Science and Engineering Research Council of Canada. The IEC analyses were performed by F. Huppertz.

Keywords

  • Amino acids
  • Inborn errors

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