1.0 2011-09-21 00:37:15 UTC 2015-07-21 06:57:57 UTC FDB023948 Nervonyl carnitine Nervonyl carnitine 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.) [HMDB] C6H16N 102.1979 102.128274517 trimethyl(propyl)azanium nervonyl carnitine CCC[N+](C)(C)C InChI=1S/C6H16N/c1-5-6-7(2,3)4/h5-6H2,1-4H3/q+1 GSBKRFGXEJLVMI-UHFFFAOYSA-N belongs to the class of organic compounds known as tetraalkylammonium salts. These are organonitrogen compounds containing a quaternary ammonium substituted with four alkyl chains. Tetraalkylammonium salts Organic compounds Organic nitrogen compounds Organonitrogen compounds Quaternary ammonium salts Aliphatic acyclic compounds Amines Hydrocarbon derivatives Organic cations Organic salts Organopnictogen compounds Aliphatic acyclic compound Amine Hydrocarbon derivative Organic cation Organic salt Organopnictogen compound Tetraalkylammonium salt Solid logp -3.48 ALOGPS logs -4.08 ALOGPS solubility 1.14e-02 g/l ALOGPS logp -3.1 ChemAxon iupac trimethyl(propyl)azanium ChemAxon average_mass 102.1979 ChemAxon mono_mass 102.128274517 ChemAxon smiles CCC[N+](C)(C)C ChemAxon formula C6H16N ChemAxon inchi InChI=1S/C6H16N/c1-5-6-7(2,3)4/h5-6H2,1-4H3/q+1 ChemAxon inchikey GSBKRFGXEJLVMI-UHFFFAOYSA-N ChemAxon polar_surface_area 0 ChemAxon refractivity 45.17 ChemAxon polarizability 13.63 ChemAxon rotatable_bond_count 2 ChemAxon acceptor_count 0 ChemAxon donor_count 0 ChemAxon physiological_charge 1 ChemAxon formal_charge 1 ChemAxon Specdb::CMs 15101 Specdb::MsMs 299503 Specdb::MsMs 299504 Specdb::MsMs 299505 HMDB06509 #<Reference:0x000055ce3259e830> Anatidae Type 1 specific Anatidae 8830 Beefalo Type 1 specific Bos taurus X Bison bison 297284 Bison Type 1 specific Bison bison 9901 Buffalo Type 1 specific Bubalus bubalis 89462 Cattle (Beef, Veal) Type 1 specific Bos taurus 9913 Chicken Type 1 specific Gallus gallus 9031 Columbidae (Dove, Pigeon) Type 1 specific Columbidae 8930 Deer Type 1 specific Cervidae 9850 Domestic goat Type 1 specific Capra aegagrus hircus 9925 Domestic pig Type 1 specific Sus scrofa domestica 9825 Elk Type 1 specific Cervus canadensis 1574408 Emu Type 1 specific Dromaius novaehollandiae 8790 European rabbit Type 1 specific Oryctolagus 9984 Greylag goose Type 1 specific Anser anser 8843 Guinea hen Type 1 specific Numida meleagris 8996 Horse Type 1 specific Equus caballus 9796 Mallard duck Type 1 specific Anas platyrhynchos 8839 Mountain hare Type 1 specific Lepus timidus 62621 Mule deer Type 1 specific Odocoileus 9871 Ostrich Type 1 specific Struthio camelus 8801 Pheasant Type 1 specific Phasianus colchicus 9054 Quail Type 1 specific Phasianidae 9005 Rabbit Type 1 specific Leporidae 9979 Rock ptarmigan Type 1 specific Lagopus muta 64668 Sheep (Mutton, Lamb) Type 1 specific Ovis aries 9940 Squab Type 1 specific Columba 8931 Turkey Type 1 specific Meleagris gallopavo 9103 Velvet duck Type 1 specific Melanitta fusca 371864 Wild boar Type 1 specific Sus scrofa 9823