1.0 2011-09-21 00:35:30 UTC 2015-07-21 06:57:53 UTC FDB023834 4,8-Dimethylnonanoyl carnitine 4,8 dimethylnonanoyl carnitine is an intermediate in phytanic and pristanic acid metabolism. Both phytanic acid and pristanic acid are initially oxidized in peroxisomes to 4,8-dimethylnonanoyl-CoA, which is then converted to to 4,8-dimethylnonanoyl carnitine (presumably by peroxisomal carnitine octanoyltransferase), and exported to the mitochondrion. After transport across the mitochondrial membrane and transfer of the acylgroup to coenzyme A, further oxidation to 2,6-dimethylheptanoyl-CoA occurs (PMID: 9469587). 4,8 dimethylnonanoyl carnitine is not a substrate for carnitine acetyltransferase, another acyltransferase localized in peroxisomes, which catalyzes the formation of carnitine esters of the other products of pristanic acid beta-oxidation, namely acetyl-CoA and propionyl-CoA. (PMID: 10486279). Earlier studies have shown that pristanic acid undergoes three cycles of beta-oxidation in peroxisomes to produce 4,8-dimethylnonanoyl-CoA (DMN-CoA) which is then transported to the mitochondria for full oxidation to CO(2) and H(2)O. In principle, this can be done via two different mechanisms in which DMN-CoA is either converted into the corresponding carnitine ester or hydrolyzed to 4,8-dimethylnonanoic acid plus CoASH.(PMID: 11785945). Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) and pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) are branched-chain fatty acids that are constituents of the human diet. As phytanic acid possesses a beta-methyl group, it cannot be degraded by beta-oxidation. Instead, phytanic acid is first degraded by alpha-oxidation, yielding pristanic acid, which is subsequently degraded by beta-oxidation. Phytanic acid alpha-oxidation is thought to occur partly, and pristanic acid beta-oxidation exclusively, in peroxisomes. Accumulation of phytanic acid and pristanic acid is found in blood and tissues of patients affected with generalized peroxisomal disorders. [HMDB] 4,8-Dimethylnonanoylcarnitine C18H35NO4 329.4748 329.256608613 3-[(4,8-dimethylnonanoyl)oxy]-4-(trimethylazaniumyl)butanoate 4,8-dimethylnonanoylcarnitine CC(C)CCCC(C)CCC(=O)OC(CC([O-])=O)C[N+](C)(C)C InChI=1S/C18H35NO4/c1-14(2)8-7-9-15(3)10-11-18(22)23-16(12-17(20)21)13-19(4,5)6/h14-16H,7-13H2,1-6H3 DDTDJDZHDFMZED-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 Branched fatty acids 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 Branched fatty acid 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 ammonium betaine carboxylic ester Solid logp -0.61 ALOGPS logs -6.21 ALOGPS solubility 2.36e-04 g/l ALOGPS logp -0.5 ChemAxon pka_strongest_acidic 4.29 ChemAxon pka_strongest_basic -7.1 ChemAxon iupac 3-[(4,8-dimethylnonanoyl)oxy]-4-(trimethylazaniumyl)butanoate ChemAxon average_mass 329.4748 ChemAxon mono_mass 329.256608613 ChemAxon smiles CC(C)CCCC(C)CCC(=O)OC(CC([O-])=O)C[N+](C)(C)C ChemAxon formula C18H35NO4 ChemAxon inchi InChI=1S/C18H35NO4/c1-14(2)8-7-9-15(3)10-11-18(22)23-16(12-17(20)21)13-19(4,5)6/h14-16H,7-13H2,1-6H3 ChemAxon inchikey DDTDJDZHDFMZED-UHFFFAOYSA-N ChemAxon polar_surface_area 66.43 ChemAxon refractivity 113.97 ChemAxon polarizability 38.58 ChemAxon rotatable_bond_count 13 ChemAxon acceptor_count 3 ChemAxon donor_count 0 ChemAxon physiological_charge 0 ChemAxon formal_charge 0 ChemAxon Oxidation of Branched Chain Fatty Acids SMP00030 Specdb::CMs 17317 Specdb::CMs 167834 Specdb::NmrOneD 67812 Specdb::NmrOneD 67813 Specdb::NmrOneD 67814 Specdb::NmrOneD 67815 Specdb::NmrOneD 67816 Specdb::NmrOneD 67817 Specdb::NmrOneD 67818 Specdb::NmrOneD 67819 Specdb::NmrOneD 67820 Specdb::NmrOneD 67821 Specdb::NmrOneD 67822 Specdb::NmrOneD 67823 Specdb::NmrOneD 67824 Specdb::NmrOneD 67825 Specdb::NmrOneD 67826 Specdb::NmrOneD 67827 Specdb::NmrOneD 67828 Specdb::NmrOneD 67829 Specdb::NmrOneD 67830 Specdb::NmrOneD 67831 Specdb::MsMs 304300 Specdb::MsMs 304301 Specdb::MsMs 304302 Specdb::MsMs 347446 Specdb::MsMs 347447 Specdb::MsMs 347448 Specdb::MsMs 2862664 Specdb::MsMs 2862665 Specdb::MsMs 2862666 HMDB06202 #<Reference:0x000055ce32561930> #<Reference:0x000055ce32561778> #<Reference:0x000055ce325615c0> 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 Carnitine O-palmitoyltransferase 2, mitochondrial P23786 CPT2 Mitochondrial carnitine/acylcarnitine carrier protein O43772 SLC25A20 Peroxisomal carnitine O-octanoyltransferase Q9UKG9 CROT