Abstract
Acylcarnitines are fatty acyl esters of L-carnitine and facilitate the entry of long-chain fatty acids into mitochondria via the carnitine shuttle, where they are metabolized via ß-oxidation. Alterations of acylcarnitine species can be diagnostic for fatty acid oxidation disorders and organic aciduria and are thus frequently used to screen newborns. Only a subfraction of all known acylcarnitines is thereby monitored and quantified. Therefore, a method for the simultaneous fast and robust detection of all known acylcarnitines was developed using a single concise liquid chromatography mass spectrometry (LC-MS) approach. Derivatization by 3-nitrophenylhydrazine increased the signal intensity of the acylcarnitines and a linear elution from a reversed phase column was observed that was dependent on the length of the carbon chain. This allowed a precise prediction of the exact elution time for each acylcarnitine class, which depended solely on the chemical nature of the carbon chain. This method can be further used to screen for yet unknown acylcarnitine species and adds a layer of confidence for their correct identification. Altogether 123 acylcarnitines species were used to establish a targeted low-resolution LC-MS method. The method was applied to acylcarnitine profiling in several mouse tissues and fluids, in order to identify large differences in the quantity and composition of acylcarnitines.
Highlights
Acyl-L-carnitines are acetylated forms of L-carnitine derived from the breakdown of amino and fatty acids [1]
The acetyl group is thereby transferred from acetyl-CoA to carnitine, resulting in CoA and acylcarnitines, which play a pivotal role in facilitating the movement of acetylCoA into the matrices of mitochondria during the oxidation of fatty acids
Gene mutations in proteins involved in these biochemical processes can lead to alterations in the acylcarnitine profile, indicative of fatty acid oxidation and organic acid metabolism disorders
Summary
Acyl-L-carnitines are acetylated forms of L-carnitine derived from the breakdown of amino and fatty acids [1]. The acetyl group is thereby transferred from acetyl-CoA to carnitine, resulting in CoA and acylcarnitines, which play a pivotal role in facilitating the movement of acetylCoA into the matrices of mitochondria during the oxidation of fatty acids. Gene mutations in proteins involved in these biochemical processes can lead to alterations in the acylcarnitine profile, indicative of fatty acid oxidation and organic acid metabolism disorders. The most common of these alterations is the accumulation of the C2-C18 acyl-CoA species, which are substrates for one of several carnitine acyl-CoA transferases [2]. Many gene mutations are directly associated with specific increases or decreases in acylcarnitine species, as summarized in several reviews [2, 3]. A onedimensional separation of acylcarnitines is commonly done by reversed phase [4,5,6] or HILIC
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
