Synonyms: (2R)-3-Carboxy-N,N,N-trimethyl-2-[(1-oxohexyl)oxy]-1-propanaminium inner salt; (R)-Caproylcarnitine; n-Hexanoyl-L-carnitine; C6-Carnitine
Molecular Formula: C13H25NO4
Molecular Weight: 259.34
Linear Structural Formula: C13H25NO4
Purity: >=97.0% (TLC)
Storage: 2-8C
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.