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Record Information
Version4.0
StatusExpected but not Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-10-23 19:03:50 UTC
HMDB IDHMDB0001359
Secondary Accession Numbers
  • HMDB01359
  • HMDB06504
Metabolite Identification
Common NamePhytanoyl-CoA
DescriptionPhytanoyl 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 ).
Structure
Thumb
Synonyms
ValueSource
3,7,11,15-Tetramethyl hexadecanoyl CoAHMDB
3,7,11,15-Tetramethyl hexadecanoyl coenzyme AHMDB
Phytanoyl CoAHMDB
Phytanoyl coenzyme AHMDB
Phytanoyl-coenzyme AHMDB
Phytanyl CoAHMDB
Phytanyl coenzyme AHMDB
Chemical FormulaC41H74N7O17P3S
Average Molecular Weight1062.049
Monoisotopic Molecular Weight1061.407474203
IUPAC Name{[(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
Traditional Name[(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 acid
CAS Registry Number146622-45-9
SMILES
CC(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=C2N
InChI Identifier
InChI=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/s1
InChI KeyNRJQGHHZMSOUEN-IYJVDCLDSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities 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.
KingdomChemical entities
Super ClassOrganic compounds
ClassLipids and lipid-like molecules
Sub ClassFatty Acyls
Direct ParentLong-chain fatty acyl CoAs
Alternative Parents
Substituents
  • Coenzyme a or derivatives
  • Purine ribonucleoside 3',5'-bisphosphate
  • Purine ribonucleoside bisphosphate
  • Purine ribonucleoside diphosphate
  • Diterpenoid
  • Pentose phosphate
  • Ribonucleoside 3'-phosphate
  • Pentose-5-phosphate
  • Beta amino acid or derivatives
  • Glycosyl compound
  • N-glycosyl compound
  • Monosaccharide phosphate
  • Organic pyrophosphate
  • 6-aminopurine
  • Pentose monosaccharide
  • Imidazopyrimidine
  • Purine
  • Monoalkyl phosphate
  • Aminopyrimidine
  • Fatty amide
  • Alkyl phosphate
  • Monosaccharide
  • N-acyl-amine
  • N-substituted imidazole
  • Organic phosphoric acid derivative
  • Imidolactam
  • Phosphoric acid ester
  • Primary aromatic amine
  • Pyrimidine
  • Oxolane
  • Azole
  • Imidazole
  • Heteroaromatic compound
  • Amino acid or derivatives
  • Thiocarboxylic acid ester
  • Carboxamide group
  • Secondary carboxylic acid amide
  • Carbothioic s-ester
  • Secondary alcohol
  • Thiocarboxylic acid or derivatives
  • Sulfenyl compound
  • Organoheterocyclic compound
  • Azacycle
  • Oxacycle
  • Carboxylic acid derivative
  • Hydrocarbon derivative
  • Organic oxygen compound
  • Organic nitrogen compound
  • Alcohol
  • Amine
  • Carbonyl group
  • Organonitrogen compound
  • Organooxygen compound
  • Organosulfur compound
  • Organopnictogen compound
  • Organic oxide
  • Primary amine
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic heteropolycyclic compounds
External DescriptorsNot Available
Ontology
Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

Source:

  Biological:

    Animal:

Route of exposure:

  Enteral:

Process

Naturally occurring process:

  Biological process:

    Biochemical pathway:

    Cellular process:

    Biochemical process:

    Chemical reaction:

Role

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.77 g/LALOGPS
logP3ALOGPS
logP0.65ChemAxon
logS-3.1ALOGPS
pKa (Strongest Acidic)0.83ChemAxon
pKa (Strongest Basic)4.95ChemAxon
Physiological Charge-4ChemAxon
Hydrogen Acceptor Count17ChemAxon
Hydrogen Donor Count9ChemAxon
Polar Surface Area363.63 ŲChemAxon
Rotatable Bond Count34ChemAxon
Refractivity254.84 m³·mol⁻¹ChemAxon
Polarizability106.07 ųChemAxon
Number of Rings3ChemAxon
Bioavailability0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-000i-4901110200-c8e2fb3674f9f0da45d7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-000i-1912130000-09f25ff6c40daa790a9cView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-000i-1900010000-5ec62b87df52230c4540View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-01rx-9652431500-04d5c22d9e4fc31c0be8View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-001l-5920210100-9766416a475cedb2730dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-057i-5900100000-b16bf5536d8209fb8b47View in MoNA
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Peroxisome
Biofluid LocationsNot Available
Tissue LocationNot Available
Pathways
NameSMPDB/PathwhizKEGG
Oxidation of Branched Chain Fatty AcidsPw000155Pw000155 greyscalePw000155 simpleNot Available
Phytanic Acid Peroxisomal OxidationPw000041Pw000041 greyscalePw000041 simpleNot Available
Refsum DiseasePw000122Pw000122 greyscalePw000122 simpleNot Available
Displaying all 3 entries
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022577
KNApSAcK IDNot Available
Chemspider ID388712
KEGG Compound IDC02060
BioCyc IDCPD-206
BiGG ID2364522
Wikipedia LinkNot Available
METLIN ID6189
PubChem Compound439640
PDB IDNot Available
ChEBI ID15538
References
Synthesis ReferenceKershaw, N. J.; Mukherji, M.; MacKinnon, C. H.; Claridge, T. D. W.; Odell, B.; Wierzbicki, A. S.; Lloyd, M. D.; Schofield, C. J. Studies on phytanoyl-CoA 2-hydroxylase and synthesis of phytanoyl-Coenzyme A. Bioorganic & Medicinal Chemistry Letters (2001),
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Bunik VI, Raddatz G, Wanders RJ, Reiser G: Brain pyruvate and 2-oxoglutarate dehydrogenase complexes are mitochondrial targets of the CoA ester of the Refsum disease marker phytanic acid. FEBS Lett. 2006 Jun 12;580(14):3551-7. Epub 2006 May 24. [PubMed:16737698 ]
  2. Schofield CJ, McDonough MA: Structural and mechanistic studies on the peroxisomal oxygenase phytanoyl-CoA 2-hydroxylase (PhyH). Biochem Soc Trans. 2007 Nov;35(Pt 5):870-5. [PubMed:17956235 ]
  3. Hostetler HA, Kier AB, Schroeder F: Very-long-chain and branched-chain fatty acyl-CoAs are high affinity ligands for the peroxisome proliferator-activated receptor alpha (PPARalpha). Biochemistry. 2006 Jun 20;45(24):7669-81. [PubMed:16768463 ]

Enzymes

General function:
Involved in acyltransferase activity
Specific function:
Carnitine acetylase is specific for short chain fatty acids. Carnitine acetylase seems to affect the flux through the pyruvate dehydrogenase complex. It may be involved as well in the transport of acetyl-CoA into mitochondria.
Gene Name:
CRAT
Uniprot ID:
P43155
Molecular weight:
70875.095
General function:
Involved in electron carrier activity
Specific function:
Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
Gene Name:
PHYH
Uniprot ID:
O14832
Molecular weight:
38538.065
Reactions
Phytanoyl-CoA + Oxoglutaric acid + Oxygen → 2-Hydroxyphytanoyl-CoA + Succinic acid + CO(2)details
General function:
Involved in catalytic activity
Specific function:
Activation of long-chain fatty acids for both synthesis of cellular lipids, and degradation via beta-oxidation. Preferentially uses palmitoleate, oleate and linoleate.
Gene Name:
ACSL1
Uniprot ID:
P33121
Molecular weight:
77942.685