1.02011-09-21 00:16:12 UTC2015-07-21 06:57:16 UTCFDB022577Phytanoyl-CoAPhytanoyl CoA is a coenzyme A derivative of phytanic acid. Phytanic acid is present in human diet or in animal tissues where it may be derived from chlorophyll in plant extracts. Specifically it is an epimeric metabolite of the isoprenoid side chain of chlorophyll. Owing to the presence of its epimeric beta-methyl group, phytanic acid cannot be metabolized by beta-oxidation. Instead, it is metabolized in peroxisomes via alpha-oxidation to give pristanic acid, which is then oxidized by beta-oxidation. PhyH (phytanoyl-CoA 2-hydroxylase) catalyses hydroxylation of phytanoyl-CoA. Mutations of PhyH can lead to phytanic acid accumulation. High levels of phytanic acid are found in patients suffering from Refsum's syndrome. This inherited neurological disorder is characterized by an accumulation of phytanic acid in blood and tissues. Clinically it is characterized by adult onset retinitis pigmentosa, anosmia, sensory neuropathy, and phytanic acidaemia. This disorder has been found to be related to deficiency in the α-oxidation pathway in the liver. (PMID: 17956235). Phytanoyl CoA and other branched-chain fatty acid CoA products are potent inducers of the peroxisome proliferator-activated receptor PPARalpha, a nuclear receptor that enhances transcription of peroxisomal enzymes mediating beta-oxidation of these potentially toxic fatty acids (PMID: 16768463). Pyruvate dehydrogenase and 2-oxoglutarate dehydrogenase are strongly inhibited by phytanoyl-CoA. Decreased activity of these important mitochondrial metabolism complexes might therefore contribute to neurological symptoms upon accumulation of phytanic acid in Refsum disease (PMID: 16737698). [HMDB]3,7,11,15-tetramethyl hexadecanoyl CoA3,7,11,15-tetramethyl hexadecanoyl coenzyme APhytanoyl CoAPhytanoyl Coenzyme Aphytanoyl-CoAPhytanoyl-coenzyme APhytanyl CoAPhytanyl coenzyme AC41H74N7O17P3S1062.0491061.407474203{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[3-hydroxy-2,2-dimethyl-3-({2-[(2-{[(3S,7R,11R)-3,7,11,15-tetramethylhexadecanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[({hydroxy[hydroxy(3-hydroxy-2,2-dimethyl-3-({2-[(2-{[(3S,7R,11R)-3,7,11,15-tetramethylhexadecanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)propoxy)phosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid146622-45-9CC(C)CCC[C@@H](C)CCC[C@@H](C)CCC[C@H](C)CC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NInChI=1S/C41H74N7O17P3S/c1-26(2)11-8-12-27(3)13-9-14-28(4)15-10-16-29(5)21-32(50)69-20-19-43-31(49)17-18-44-39(53)36(52)41(6,7)23-62-68(59,60)65-67(57,58)61-22-30-35(64-66(54,55)56)34(51)40(63-30)48-25-47-33-37(42)45-24-46-38(33)48/h24-30,34-36,40,51-52H,8-23H2,1-7H3,(H,43,49)(H,44,53)(H,57,58)(H,59,60)(H2,42,45,46)(H2,54,55,56)/t27-,28-,29+,30-,34-,35-,36?,40-/m1/s1NRJQGHHZMSOUEN-IYJVDCLDSA-N belongs to the class of organic compounds known as long-chain fatty acyl coas. These are acyl CoAs where the group acylated to the coenzyme A moiety is a long aliphatic chain of 13 to 21 carbon atoms.Long-chain fatty acyl CoAsOrganic compoundsLipids and lipid-like moleculesFatty AcylsFatty acyl thioestersAromatic heteropolycyclic compounds2,3,4-saturated fatty acyl CoAs6-aminopurinesAminopyrimidines and derivativesAzacyclic compoundsBeta amino acids and derivativesCarbonyl compoundsCarbothioic S-estersCoenzyme A and derivativesDiterpenoidsGlycosylaminesHeteroaromatic compoundsHydrocarbon derivativesImidolactamsMonoalkyl phosphatesMonosaccharide phosphatesN-acyl aminesN-substituted imidazolesOrganic oxidesOrganic pyrophosphatesOrganopnictogen compoundsOxacyclic compoundsPentose phosphatesPrimary aminesPurine ribonucleoside diphosphatesRibonucleoside 3'-phosphatesSecondary alcoholsSecondary carboxylic acid amidesSulfenyl compoundsTetrahydrofuransThioesters6-aminopurineAlcoholAlkyl phosphateAmineAmino acid or derivativesAminopyrimidineAromatic heteropolycyclic compoundAzacycleAzoleBeta amino acid or derivativesCarbonyl groupCarbothioic s-esterCarboxamide groupCarboxylic acid derivativeCoenzyme a or derivativesDiterpenoidFatty amideGlycosyl compoundHeteroaromatic compoundHydrocarbon derivativeImidazoleImidazopyrimidineImidolactamMonoalkyl phosphateMonosaccharideMonosaccharide phosphateN-acyl-amineN-glycosyl compoundN-substituted imidazoleOrganic nitrogen compoundOrganic oxideOrganic oxygen compoundOrganic phosphoric acid derivativeOrganic pyrophosphateOrganoheterocyclic compoundOrganonitrogen compoundOrganooxygen compoundOrganopnictogen compoundOrganosulfur compoundOxacyclePentose monosaccharidePentose phosphatePentose-5-phosphatePhosphoric acid esterPrimary aminePurinePurine ribonucleoside 3',5'-bisphosphatePurine ribonucleoside bisphosphatePurine ribonucleoside diphosphatePyrimidineRibonucleoside 3'-phosphateSecondary alcoholSecondary carboxylic acid amideSulfenyl compoundTetrahydrofuranThiocarboxylic acid esterThiocarboxylic acid or derivativesSolidlogp3.00logs-3.14solubility7.67e-01 g/llogp0.65pka_strongest_acidic0.83pka_strongest_basic4.95iupac{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[3-hydroxy-2,2-dimethyl-3-({2-[(2-{[(3S,7R,11R)-3,7,11,15-tetramethylhexadecanoyl]sulfanyl}ethyl)carbamoyl]ethyl}carbamoyl)propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acidaverage_mass1062.049mono_mass1061.407474203smilesCC(C)CCC[C@@H](C)CCC[C@@H](C)CCC[C@H](C)CC(=O)SCCNC(=O)CCNC(=O)C(O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2NformulaC41H74N7O17P3SinchiInChI=1S/C41H74N7O17P3S/c1-26(2)11-8-12-27(3)13-9-14-28(4)15-10-16-29(5)21-32(50)69-20-19-43-31(49)17-18-44-39(53)36(52)41(6,7)23-62-68(59,60)65-67(57,58)61-22-30-35(64-66(54,55)56)34(51)40(63-30)48-25-47-33-37(42)45-24-46-38(33)48/h24-30,34-36,40,51-52H,8-23H2,1-7H3,(H,43,49)(H,44,53)(H,57,58)(H,59,60)(H2,42,45,46)(H2,54,55,56)/t27-,28-,29+,30-,34-,35-,36?,40-/m1/s1inchikeyNRJQGHHZMSOUEN-IYJVDCLDSA-Npolar_surface_area363.63refractivity254.84polarizability106.07rotatable_bond_count34acceptor_count17donor_count9physiological_charge-4formal_charge0Oxidation of Branched Chain Fatty AcidsSMP00030Phytanic Acid Peroxisomal OxidationSMP00450Specdb::MsMs289984Specdb::MsMs289985Specdb::MsMs289986Specdb::MsMs329572Specdb::MsMs329573Specdb::MsMs329574Specdb::MsMs2421315Specdb::MsMs2421316Specdb::MsMs2421317Specdb::MsMs2544694Specdb::MsMs2544695Specdb::MsMs2544696HMDB0135915538#<Reference:0x0000555675a581f8>#<Reference:0x0000555675a58018>#<Reference:0x0000555675a57d98>AnatidaeType 1specificAnatidae8830BeefaloType 1specificBos taurus X Bison bison297284BisonType 1specificBison bison9901BuffaloType 1specificBubalus bubalis89462Cattle (Beef, Veal)Type 1specificBos taurus9913ChickenType 1specificGallus gallus9031Columbidae (Dove, Pigeon)Type 1specificColumbidae8930DeerType 1specificCervidae9850Domestic goatType 1specificCapra aegagrus hircus9925Domestic pigType 1specificSus scrofa domestica9825ElkType 1specificCervus canadensis1574408EmuType 1specificDromaius novaehollandiae8790European rabbitType 1specificOryctolagus9984Greylag gooseType 1specificAnser anser8843Guinea henType 1specificNumida meleagris8996HorseType 1specificEquus caballus9796Mallard duckType 1specificAnas platyrhynchos8839Mountain hareType 1specificLepus timidus62621Mule deerType 1specificOdocoileus9871OstrichType 1specificStruthio camelus8801PheasantType 1specificPhasianus colchicus9054QuailType 1specificPhasianidae9005RabbitType 1specificLeporidae9979Rock ptarmiganType 1specificLagopus muta64668Sheep (Mutton, Lamb)Type 1specificOvis aries9940SquabType 1specificColumba8931TurkeyType 1specificMeleagris gallopavo9103Velvet duckType 1specificMelanitta fusca371864Wild boarType 1specificSus scrofa9823Carnitine O-acetyltransferaseP43155CRAT