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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:04:19 UTC
HMDB IDHMDB01339
Secondary Accession NumbersNone
Metabolite Identification
Common NameGlutaryl-CoA
DescriptionGlutaryl-CoA is a substrate for 2-oxoglutarate dehydrogenase E1 component (mitochondrial), Dihydrolipoyllysine-residue succinyltransferase component of 2- oxoglutarate dehydrogenase complex (mitochondrial) and Glutaryl-CoA dehydrogenase (mitochondrial).
Structure
Thumb
Synonyms
ValueSource
Glutaryl-coenzyme AHMDB
Chemical FormulaC26H42N7O19P3S
Average Molecular Weight881.633
Monoisotopic Molecular Weight881.146902423
IUPAC Name5-{[2-(3-{3-[({[({[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy}(hydroxy)phosphoryl)oxy](hydroxy)phosphoryl}oxy)methyl]-2-hydroxy-3-methylbutanamido}propanamido)ethyl]sulfanyl}-5-oxopentanoic acid
Traditional Name5-({2-[3-(3-{[({[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-3-(phosphonooxy)oxolan-2-yl]methoxy(hydroxy)phosphoryl}oxy(hydroxy)phosphoryl)oxy]methyl}-2-hydroxy-3-methylbutanamido)propanamido]ethyl}sulfanyl)-5-oxopentanoic acid
CAS Registry Number103192-48-9
SMILES
CC(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)C(O)C(=O)NCCC(=O)NCCSC(=O)CCCC(O)=O
InChI Identifier
InChI=1S/C26H42N7O19P3S/c1-26(2,21(39)24(40)29-7-6-15(34)28-8-9-56-17(37)5-3-4-16(35)36)11-49-55(46,47)52-54(44,45)48-10-14-20(51-53(41,42)43)19(38)25(50-14)33-13-32-18-22(27)30-12-31-23(18)33/h12-14,19-21,25,38-39H,3-11H2,1-2H3,(H,28,34)(H,29,40)(H,35,36)(H,44,45)(H,46,47)(H2,27,30,31)(H2,41,42,43)/t14-,19-,20-,21?,25-/m1/s1
InChI KeyInChIKey=SYKWLIJQEHRDNH-KRPIADGTSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as 2,3,4-saturated fatty acyl coas. These are acyl-CoAs carrying a 2,3,4-saturated fatty acyl chain.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acyl thioesters
Direct Parent2,3,4-saturated fatty acyl CoAs
Alternative Parents
Substituents
  • Coenzyme a or derivatives
  • Purine ribonucleoside diphosphate
  • Purine ribonucleoside 3',5'-bisphosphate
  • N-glycosyl compound
  • Glycosyl compound
  • Beta amino acid or derivatives
  • Organic pyrophosphate
  • Monosaccharide phosphate
  • 6-aminopurine
  • Purine
  • Imidazopyrimidine
  • Monoalkyl phosphate
  • Aminopyrimidine
  • Imidolactam
  • Alkyl phosphate
  • Pyrimidine
  • Primary aromatic amine
  • Phosphoric acid ester
  • Organic phosphoric acid derivative
  • Organic phosphate
  • N-substituted imidazole
  • N-acyl-amine
  • Monosaccharide
  • Fatty amide
  • Saccharide
  • Heteroaromatic compound
  • Oxolane
  • Imidazole
  • Azole
  • Thiocarboxylic acid ester
  • Secondary carboxylic acid amide
  • Secondary alcohol
  • Carboxamide group
  • Oxacycle
  • Azacycle
  • Organoheterocyclic compound
  • Sulfenyl compound
  • Thioether
  • Thiocarboxylic acid or derivatives
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Carboxylic acid amide
  • Hydrocarbon derivative
  • Primary amine
  • Organosulfur compound
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Amine
  • Alcohol
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic heteropolycyclic compounds
External DescriptorsNot Available
Ontology
StatusExpected but not Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Component of Fatty acid metabolism
  • Component of Tryptophan metabolism
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Extracellular
  • Membrane
  • Mitochondria
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 Solubility3.62 mg/mLALOGPS
logP-0.48ALOGPS
logP-6.7ChemAxon
logS-2.4ALOGPS
pKa (Strongest Acidic)0.83ChemAxon
pKa (Strongest Basic)5.01ChemAxon
Physiological Charge-5ChemAxon
Hydrogen Acceptor Count19ChemAxon
Hydrogen Donor Count10ChemAxon
Polar Surface Area400.93 Å2ChemAxon
Rotatable Bond Count24ChemAxon
Refractivity187.7 m3·mol-1ChemAxon
Polarizability78.02 Å3ChemAxon
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-1912000130-9b9c96863df04861fbeeView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-000i-0913000000-43f2e61c32509ed14b97View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-000i-1901000000-4ff7fbc1bd490ad4c020View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-01q9-3911030350-425e74ceb7a0a4256402View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-01si-2901010010-f93605fcb2512efdd305View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-057i-6900100000-74a35eb8b1b0810315e4View in MoNA
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Mitochondria
Biofluid LocationsNot Available
Tissue Location
  • Fibroblasts
  • Liver
Pathways
NameSMPDB LinkKEGG Link
2-aminoadipic 2-oxoadipic aciduriaSMP00719Not Available
Carnitine palmitoyl transferase deficiency (I)SMP00538Not Available
Carnitine palmitoyl transferase deficiency (II)SMP00541Not Available
Ethylmalonic EncephalopathySMP00181Not Available
Fatty acid MetabolismSMP00051map00071
Glutaric Aciduria Type ISMP00185Not Available
Glutaric Aciduria Type ISMP00186Not Available
Hyperlysinemia I, FamilialSMP00527Not Available
Hyperlysinemia II or SaccharopinuriaSMP00528Not Available
Long chain acyl-CoA dehydrogenase deficiency (LCAD)SMP00539Not Available
Lysine DegradationSMP00037map00310
Medium chain acyl-coa dehydrogenase deficiency (MCAD)SMP00542Not Available
Pyridoxine dependency with seizuresSMP00571Not Available
Saccharopinuria/Hyperlysinemia IISMP00239Not Available
Short Chain Acyl CoA Dehydrogenase Deficiency (SCAD Deficiency)SMP00235Not Available
Trifunctional protein deficiencySMP00545Not Available
Very-long-chain acyl coa dehydrogenase deficiency (VLCAD)SMP00540Not Available
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 IDFDB022563
KNApSAcK IDNot Available
Chemspider ID388388
KEGG Compound IDC00527
BioCyc IDGLUTARYL-COA
BiGG ID35255
Wikipedia LinkGlutaryl-CoA
NuGOwiki LinkHMDB01339
Metagene LinkHMDB01339
METLIN ID6173
PubChem Compound439252
PDB IDNot Available
ChEBI ID15524
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Corral I, Martinez Castrillo JC, Martinez-Pardo M, Gimeno A: [Glutaric aciduria type I: diagnosis in adulthood and phenotypic variability] Neurologia. 2001 Oct;16(8):377-80. [11738016 ]
  2. Mahfoud A, Dominguez CL, Rizzo C, Ribes A: [In utero macrocephaly as clinical manifestation of glutaric aciduria type I. Report of a novel mutation] Rev Neurol. 2004 Nov 16-30;39(10):939-42. [15573311 ]
  3. Hyman DB, Tanaka K: Specific glutaryl-CoA dehydrogenating activity is deficient in cultured fibroblasts from glutaric aciduria patients. J Clin Invest. 1984 Mar;73(3):778-84. [6423663 ]

Enzymes

General function:
Involved in oxidoreductase activity
Specific function:
Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
Gene Name:
DLD
Uniprot ID:
P09622
Molecular weight:
54176.91
General function:
Involved in acyl-CoA dehydrogenase activity
Specific function:
Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive.
Gene Name:
GCDH
Uniprot ID:
Q92947
Molecular weight:
48126.715
Reactions
Glutaryl-CoA + electron-transfer flavoprotein → (E)-but-2-enoyl-CoA + CO(2) + reduced electron-transfer flavoproteindetails
Glutaryl-CoA + FAD → FADH + (E)-but-2-enoyl-CoA + Carbon dioxidedetails
Glutaryl-CoA + Electron-transferring flavoprotein → (E)-but-2-enoyl-CoA + Reduced electron-transferring flavoprotein + Carbon dioxidedetails
Glutaryl-CoA + Electron-transferring flavoprotein → Glutaconyl-CoA + Reduced electron-transferring flavoproteindetails
General function:
Involved in oxoglutarate dehydrogenase (succinyl-transferring) activity
Specific function:
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3).
Gene Name:
OGDH
Uniprot ID:
Q02218
Molecular weight:
48179.59
Reactions
Oxoadipic acid + Coenzyme A + NAD → Glutaryl-CoA + Carbon dioxide + NADH + Hydrogen Iondetails
General function:
Involved in acyltransferase activity
Specific function:
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of 3 enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3).
Gene Name:
DLST
Uniprot ID:
P36957
Molecular weight:
48754.87
Reactions
Glutaryl-CoA + Enzyme N6-(dihydrolipoyl)lysine → Coenzyme A + [Dihydrolipoyllysine-residue succinyltransferase] S-glutaryldihydrolipoyllysinedetails
General function:
Involved in oxoglutarate dehydrogenase (succinyl-transferring) activity
Specific function:
Not Available
Gene Name:
OGDHL
Uniprot ID:
Q9ULD0
Molecular weight:
Not Available
Reactions
Oxoadipic acid + Coenzyme A + NAD → Glutaryl-CoA + Carbon dioxide + NADH + Hydrogen Iondetails