Abstract
Acylcarnitines are formed by esterification between fatty acids CoA or organic acids CoA molecules and carnitine. In various fatty acids oxidation defects and organic acidurias, there is increased concentration of corresponding acylcarnitines. Abnormalities in specific acylcarnitines are used in the diagnosis of fatty acids oxidation defects and organic acidurias. Most commonly used method for the assay of acylcarnitines is HPLC-tandem mass spectrometry (HPLC/MS/MS). A HPLC/MS/MS method is described for the quantification of number of acylcarnitines. The method involves butylation of carnitine/acylcarnitines using acidified butanol, HPLC flow injection, and measurement of acylcarnitines using precursor ion scan and multiple reactions monitoring (MRM).
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Scott, D., Heese, B., Garg, U. (2016). Quantification of Free Carnitine and Acylcarnitines in Plasma or Serum Using HPLC/MS/MS. In: Garg, U. (eds) Clinical Applications of Mass Spectrometry in Biomolecular Analysis. Methods in Molecular Biology, vol 1378. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-3182-8_2
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DOI: https://doi.org/10.1007/978-1-4939-3182-8_2
Publisher Name: Humana Press, New York, NY
Print ISBN: 978-1-4939-3181-1
Online ISBN: 978-1-4939-3182-8
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