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Acylcarnitines

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

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