Identification | Back Directory | [Name]
L-Hexanoylcarnitine | [CAS]
22671-29-0 | [Synonyms]
L-Hexanoylcarnitine HEXANOYL-L-CARNITINE O-hexanoyl-L-carnitine Hexanoyl-L-carnitine >=97.0% (TLC) | [Molecular Formula]
C13H25NO4 | [MDL Number]
MFCD28580109 | [MOL File]
22671-29-0.mol |
Hazard Information | Back Directory | [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. |
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