Title

Quantification of Free Carnitine and Acylcarnitines in Plasma or Serum Using HPLC/MS/MS.

Document Type

Article

Publication Date

1-1-2016

Identifier

DOI: 10.1007/978-1-4939-3182-8_2

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).

Journal Title

Methods in molecular biology (Clifton, N.J.)

Volume

1378

First Page

11

Last Page

19

MeSH Keywords

Blood Chemical Analysis; Carnitine; Chromatography, High Pressure Liquid; Humans; Reference Standards; Tandem Mass Spectrometry

Keywords

Fatty acid oxidation defects; HPLC; Inborn error of metabolism; Inherited metabolic disorders; Mass spectrometry; Medium chain acyl-CoA dehydrogenase deficiency; Organic acidemia; Organic acidurias

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