Quantification of 3-methylglutaconic acid in urine, plasma, and amniotic fluid by isotope-dilution gas chromatography/mass spectrometry

Richard I. Kelley

Research output: Contribution to journalArticle

Abstract

A method is described for quantification of the trace metabolite, 3-methylglutaconic acid, by isotope-dilution gas chromatography/mass spectrometry using synthetic 3-[2,4,6-13C3] methylglutaconic acid. Results are shown for quantification of 3-methylglutaconic acid in plasma, urine, cerebrospinal fluid and amniotic fluid for both normal controls and patients with different forms of 3-methylglutaconic aciduria. A simple method for the synthesis and purification of 3-[2,4,6-13C3]methylglutaconic acid is also described.

Original languageEnglish (US)
Pages (from-to)157-164
Number of pages8
JournalClinica Chimica Acta
Volume220
Issue number2
DOIs
StatePublished - Nov 15 1993

Keywords

  • 3-Methylglutaconic acid
  • Inborn error of metabolism
  • Mass spectrometry
  • Stable isotope dilution

ASJC Scopus subject areas

  • Biochemistry
  • Clinical Biochemistry
  • Biochemistry, medical

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