1.0 2011-09-21 00:09:52 UTC 2015-10-09 22:33:40 UTC FDB022192 Hexanoylcarnitine Hexanoylcarnitine is an acylcarnitine. 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 reabsorption 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. (PMID: 17508264, Monatshefte fuer Chemie (2005), 136(8), 1279-1291., Int J Mass Spectrom. 1999;188:39-52.) Hexanoylcarnitine is a medium-chain acylcarnitine present in the urine of patients with medium-chain acyl-CoA dehydrogenase deficiency (PubMed ID 1635814 ) [HMDB] 3-(Hexanoyloxy)-4-(trimethylammonio)butanoate 3-(Hexanoyloxy)-4-(trimethylammonio)butanoic acid Caproylcarnitine Hexanoic acid ester with (3-carboxy-2-hydroxypropyl)trimethylammonium hydroxide inner salt Hexanoyl DL-carnitine Hexanoyl-d,l-carnitine Hexanoylcarnitine C13H25NO4 259.3419 259.178358293 3-(hexanoyloxy)-4-(trimethylazaniumyl)butanoate hexanoyl-d,l-carnitine 6418-78-6 CCCCCC(=O)OC(CC([O-])=O)C[N+](C)(C)C InChI=1S/C13H25NO4/c1-5-6-7-8-13(17)18-11(9-12(15)16)10-14(2,3)4/h11H,5-10H2,1-4H3 VVPRQWTYSNDTEA-UHFFFAOYSA-N belongs to the class of organic compounds known as acyl carnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond. Acyl carnitines Organic compounds Lipids and lipid-like molecules Fatty Acyls Fatty acid esters Aliphatic acyclic compounds Amines Carbonyl compounds Carboxylic acid esters Carboxylic acid salts Carboxylic acids Dicarboxylic acids and derivatives Hydrocarbon derivatives Organic oxides Organic salts Organopnictogen compounds Tetraalkylammonium salts Acyl-carnitine Aliphatic acyclic compound Amine Carbonyl group Carboxylic acid Carboxylic acid derivative Carboxylic acid ester Carboxylic acid salt Dicarboxylic acid or derivatives Hydrocarbon derivative Organic nitrogen compound Organic oxide Organic oxygen compound Organic salt Organonitrogen compound Organooxygen compound Organopnictogen compound Quaternary ammonium salt Tetraalkylammonium salt O-acylcarnitine Solid logp -1.68 ALOGPS logs -4.86 ALOGPS solubility 4.30e-03 g/l ALOGPS logp -2.4 ChemAxon pka_strongest_acidic 4.22 ChemAxon pka_strongest_basic -7.1 ChemAxon iupac 3-(hexanoyloxy)-4-(trimethylazaniumyl)butanoate ChemAxon average_mass 259.3419 ChemAxon mono_mass 259.178358293 ChemAxon smiles CCCCCC(=O)OC(CC([O-])=O)C[N+](C)(C)C ChemAxon formula C13H25NO4 ChemAxon inchi InChI=1S/C13H25NO4/c1-5-6-7-8-13(17)18-11(9-12(15)16)10-14(2,3)4/h11H,5-10H2,1-4H3 ChemAxon inchikey VVPRQWTYSNDTEA-UHFFFAOYSA-N ChemAxon polar_surface_area 66.43 ChemAxon refractivity 91.07 ChemAxon polarizability 29.12 ChemAxon rotatable_bond_count 10 ChemAxon acceptor_count 3 ChemAxon donor_count 0 ChemAxon physiological_charge 0 ChemAxon formal_charge 0 ChemAxon Specdb::NmrTwoD 1436 Specdb::NmrOneD 1490 Specdb::MsMs 984 Specdb::MsMs 985 Specdb::MsMs 986 HMDB00705 #<Reference:0x000055ce32cbc1d0> #<Reference:0x000055ce32cbc018> #<Reference:0x000055ce32cbbed8> #<Reference:0x000055ce32cbbc80> #<Reference:0x000055ce32cbbaa0> #<Reference:0x000055ce32cbb780> #<Reference:0x000055ce32cbb500> #<Reference:0x000055ce32cbb2f8> #<Reference:0x000055ce32cbaec0> #<Reference:0x000055ce32cbace0> #<Reference:0x000055ce32cbaab0> #<Reference:0x000055ce32cba628> #<Reference:0x000055ce32cba420> #<Reference:0x000055ce32cba128> #<Reference:0x000055ce32cb9ea8> #<Reference:0x000055ce32cb9c28> #<Reference:0x000055ce32cb9868>