L-Hexanoylcarnitine

Uses

Hexanoyl-L-carnitine may be used as an analytical reference standard for the quantification of the analyte in dried blood spots using high-performance liquid chromatography coupled to electrospray ionization tandem mass spectrometry (HPLC-ESI-MS/MS). Hexanoyl-L-carnitine may also be used as an analytical standard for the separation and identification of underivatized hexanoyl-L-carnitine in human plasma samples using HPLC-MS/MS.

Definition

ChEBI: An O-hexanoylcarnitine that has L configuration.

General Description

Hexanoyl-L-carnitine belongs to the class of straight-chain acylcarnitines, which are important biomarkers in the diagnosis of carnitine deficiency.

Biochem/physiol Actions

Numerous disorders have been described that lead to disturbances in energy production and in intermediary metabolism in the organism which are characterized by the production and excretion of unusual acylcarnitines. A mutation in the gene coding for carnitine-acylcarnitine translocase or the OCTN2 transporter aetiologically causes a carnitine deficiency that results in poor intestinal absorption of dietary L-carnitine, its impaired re-absorption by the kidney and, consequently, in increased urinary loss of L-carnitine. Determination of the qualitative pattern of acylcarnitines can be of diagnostic and therapeutic importance. The betaine structure of carnitine requires special analytical procedures for recording. The ionic nature of L-carnitine causes a high water solubility which decreases with increasing chain length of the ester group in the acylcarnitines. Therefore, the distribution of L-carnitine and acylcarnitines in various organs is defined by their function and their physico-chemical properties as well. High performance liquid chromatography (HPLC) permits screening for free and total carnitine, as well as complete quantitative acylcarnitine determination, including the long-chain acylcarnitine profile.