Interconversion of acyl-CoA esters and acylcarnitines plays a key role in the transport process of fatty acids across the mitochondrial membrane. Acylcarnitine profiling in plasma has become indispensable for the diagnosis of a series of fatty acid ß-oxidation deficiencies, and of defects specifically related to the membrane transport process. Increasingly, acylcarnitine profiling in blood spots by MS/MS is included in national neonatal screening programs
Standard: acetyl-L-carnitine.HCl
Labeled standard: [2H3]acetyl-L-carnitine.HCl
Standard: propionyl-L-carnitine.HCl
Labeled standard: [3,3,3-2H3]propionyl-L-carnitine.HCl
Standard:butyryl-L-carnitine.HCl
Labeled standard: [4,4,4-2H3]butyryl-L-carnitine.HCl
Standard: isobutyryl-L-carnitine.HCl
Labeled standard: [2H7]isobutyryl-L-carnitine.HCl
Standard: pivaloyl-L-carnitine.HCl
Standard: valeryl-L-carnitine.HCl
Labeled standard: [5,5,5-2H3]valeryl-L-carnitine.HCl
Standard: isovaleryl-L-carnitine.HCl
Labeled standard: [2H9]isovaleryl-L-carnitine.HCl
Standard: 2-methylbutyryl-L-carnitine.HCl
Labeled standard: 2-[2H3]methylbutyryl-L-carnitine.HCl
Standard: hexanoyl-L-carnitine.HCL
Labeled standard: [6,6,6-2H3]hexanoyl-L-carnitine.HCl
Standard: octanoyl-L-carnitine.HCl
Labeled standard: [8,8,8-2H3]octanoyl-L-carnitine.HCl
Standard: decanoyl-L-carnitine.HCl
Labeled standard: [10,10,10-2H3]decanoyl-L-carnitine.HCl
Standard: dodecanoyl-L-carnitine.HCl
Labeled standard: [12,12,12-2H3]dodecanoyl-L-carnitine.HCl
Standard: tetradecanoyl-L-carnitine.HCl
Labeled standard: [14,14,14-2H3]tetradecanoyl-L-carnitine.HCl
Standard: hexadecanoyl-L-carnitine.HCl
Labeled standard: [16,16,16-2H3]hexadecanoyl-L-carnitine.HCl
Standard: octadecanoyl-L-carnitine.HCl
Labeled standard: [18,18,18-2H3]octadecanoyl-L-carnitine.HCL
Synthesis and application:
CG Costa, EA Struys, A Bootsma, HJ ten Brink, L Dorland, I Tavares de Almeida, M Duran, C Jakobs. Quantitative analysis of plasma acylcarnitines using gas chromatography chemical ionization mass fragmentography. J Lipid Res 38 (1997) 173-182.
Carnitine-labelled standards:
[methyl-2H3]-L-carnitine.HCl
[trimethyl-2H9]-D,L-carnitine.HCL
[4,4,4-2H3]butyryl -[methyl-2H3]-L-carnitine.HCl
[8,8,8-2H3]octanoyl -[methyl-2H3]-L-carnitine.HCl
[16,16,16-2H3]hexadecanoyl -[methyl-2H3]-L-carnitine.HCl