Urinary succinylacetone analysis by gas chromatography-mass spectrometry (GC-MS)

Hongjie Chen, Chunli Yu

Research output: Chapter in Book/Report/Conference proceedingChapterpeer-review

2 Scopus citations

Abstract

Succinylacetone (SA) is used for the diagnosis and monitoring of patients with tyrosinemia type I (Tyr I). SA is exclusively elevated in blood and urine of patients with Tyr I. As urinary SA concentration is much higher than blood, SA is usually tested in urine samples. Urinary SA quantitation by gas chromatography mass spectrometry (GC-MS) is described in this chapter. The urine sample in the amount of 1 μmol creatinine is used for testing. 3,4,5,6,7-13C5-succinylacetone (13C5-SA) is used as an internal standard (IS). SA and 13C5-SA are oximated and extracted from urine with organic solvents, and then derivatized to form trimethylsilane (TMS) derivatives. TMS derivatives of SA and 13C5-SA are detected and quantified by GC-MS using selective ion monitoring (SIM). The assay is linear from 0.05 to 450 mmol/mol creatinine to cover the broad range of urinary SA concentrations.

Original languageEnglish
Title of host publicationMethods in Molecular Biology
PublisherHumana Press Inc.
Pages281-290
Number of pages10
DOIs
StatePublished - 2016

Publication series

NameMethods in Molecular Biology
Volume1378
ISSN (Print)1064-3745

Keywords

  • Gas chromatography
  • Mass spectrometry
  • Succinylacetone
  • Tyrosinemia I

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