Uses
A potential prodrug, via the carnitine transporter OCTN2 and the amino acid transporter ATB0,+
Uses
Butyryl-L-carnitine may be used as an analytical reference standard for the separation and identification of underivatized butyryl-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 biological samples using chromatography-based techniques.
Definition
ChEBI: An optically active form of O-butanoylcarnitine having L-configuration.
General Description
Butyryl-L-carnitine is a naturally occurring compound belonging to the acylcarnitine class of compounds as listed in the human metabolome database (HMBD). These compounds are reported to be used as biomarkers in the diagnosis of carnitine deficiency. It acts as a carrier of long chain acyl groups from activated fatty acids across the inner mitochondrial membrane into the mitochondrial matrix where they undergo β-oxidation to acetyl CoA to obtain usable energy via the citric acid cycle.
Biochem/physiol Actions
Butyrylcarnitine is elevated in patients with Acyl-CoA dehydrogenase, short-chain (SCAD) deficiency, in infants with acute acidosis and generalized muscle weakness and in middle-aged patients with chronic myopathy localized in muscle; very long chain Acyl-CoA dehydrogenase deficiency, and celiac disease.
