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Identification of urinary acylcarnitines using gas chromatography-mass spectrometry: preliminary clinical applications.

Abstract
Many disorders of organic acid metabolism are associated with abnormalities in the levels of acylcarnitines excreted in urine. Profiling of urinary acylcarnitines allows diagnosis and characterisation of many acidurias and acidemias, monitoring dietary treatment of such patients, and elucidation of the metabolism of some exogenous acidic compounds. Urine (ca. 0.5 ml) was subjected to a simple work-up by ion-exchange chromatography, and the isolated acylcarnitines were derivatized by cyclization in 35 min to give volatile lactones that are compatible with gas chromatography-mass spectrometry using electron or chemical ionization. The feasibility of this new and affordable procedure has been confirmed by identifying urinary acylcarnitines in cases of medium-chain acyl-coenzyme A dehydrogenase deficiency, propionic acidemia and isovaleric acidemia.
AuthorsS Lowes, M E Rose, G A Mills, R J Pollitt
JournalJournal of chromatography (J Chromatogr) Vol. 577 Issue 2 Pg. 205-14 (Jun 10 1992) Netherlands
PMID1400753 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Carnitine
Topics
  • Acylation
  • Carnitine (analogs & derivatives, urine)
  • Gas Chromatography-Mass Spectrometry
  • Humans
  • Infant
  • Metabolism, Inborn Errors (diagnosis, urine)

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