Isobutyryl-L-carnitine may be used as an analytical reference standard for the separation and identification of underivatized isobutyryl-L-carnitine in human plasma samples using high-performance liquid chromatography coupled to tandem mass spectrometry (HPLC-MS/MS). It may also be used as an analytical reference standard for the quantification of the analyte in human urine samples using liquid chromatography/electrospray ionization tandem mass spectrometry (LC/ESI-MS/MS).
ChEBI: An optically active form of O-isobutyrylcarnitine having (R)-configuration.
Isobutyryl-L-carnitine belongs to the acylcarnitines class of compounds. These compounds are produced from L-carnitine, which plays an important role in fatty acid metabolic pathways. The functions and roles of acyl-L-carnitines in various tissues like brain, heart, and muscle continue to attract much interest.
Isobutyryl-L-carnitine is a product of the acyl-CoA dehydrogenases (ACADs), which are a group of mitochondrial enzymes involved in the metabolism of fatty acids or branched-chain amino acids.