You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.

Showing metabocard for Propionyl-CoA (HMDB0001275)

Jump To Section:
IdentificationTaxonomyOntologyPhysical propertiesSpectraBiological propertiesConcentrationsLinksReferencesenzymes (121)transporters (1) Show 122 proteinsXML
enzymes (121)transporters (1) Show 122 proteins
Record Information
Version4.0
StatusExpected but not Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-06-23 16:12:53 UTC
HMDB IDHMDB0001275
Secondary Accession Numbers
  • HMDB01275
Metabolite Identification
Common NamePropionyl-CoA
DescriptionPropionyl-CoA is an intermediate in the metabolism of propanoate. Propionic aciduria is caused by an autosomal recessive disorder of propionyl coenzyme A (CoA) carboxylase deficiency (EC 6.4.1.3). In propionic aciduria, propionyl CoA accumulates within the mitochondria in massive quantities; free carnitine is then esterified, creating propionyl carnitine, which is then excreted in the urine. Because the supply of carnitine in the diet and from synthesis is limited, such patients readily develop carnitine deficiency as a result of the increased loss of acylcarnitine derivatives. This condition demands supplementation of free carnitine above the normal dietary intake to continue to remove (detoxify) the accumulating organic acids. Propionyl-CoA is a substrate for Acyl-CoA dehydrogenase (medium-chain specific, mitochondrial), Acetyl-coenzyme A synthetase 2-like (mitochondrial), Propionyl-CoA carboxylase alpha chain (mitochondrial), Methylmalonate-semialdehyde dehydrogenase (mitochondrial), Trifunctional enzyme beta subunit (mitochondrial), 3-ketoacyl-CoA thiolase (peroxisomal), Acyl-CoA dehydrogenase (long-chain specific, mitochondrial), Malonyl-CoA decarboxylase (mitochondrial), Acetyl-coenzyme A synthetase (cytoplasmic), 3-ketoacyl-CoA thiolase (mitochondrial) and Propionyl-CoA carboxylase beta chain (mitochondrial). (PMID: 10650319 ).
Structure
Data?1592928773
MOLSDFPDBSMILESInChI
Synonyms
ValueSource
Propanoyl-coenzyme AChEBI
Propionyl coenzyme AChEBI
Propionyl-coenzyme AChEBI
S-Propanoyl-CoAChEBI
S-Propanoyl-coenzyme AChEBI
S-Propionyl-coenzym-aChEBI
S-Propionylcoenzyme AChEBI
Propanoyl-CoAChEBI
Propionyl CoAHMDB
Propionyl-CoAHMDB
n-Propionyl CoAHMDB
Chemical FormulaC24H40N7O17P3S
Average Molecular Weight823.597
Monoisotopic Molecular Weight823.141423115
IUPAC Name{[(2R,3S,4R,5R)-5-(6-amino-9H-purin-9-yl)-4-hydroxy-2-({[hydroxy({hydroxy[(3R)-3-hydroxy-2,2-dimethyl-3-[(2-{[2-(propanoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]propoxy]phosphoryl}oxy)phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid
Traditional Namepropionyl-coa
CAS Registry Number317-66-8
SMILES
CCC(=O)SCCNC(=O)CCNC(=O)[C@H](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/C24H40N7O17P3S/c1-4-15(33)52-8-7-26-14(32)5-6-27-22(36)19(35)24(2,3)10-45-51(42,43)48-50(40,41)44-9-13-18(47-49(37,38)39)17(34)23(46-13)31-12-30-16-20(25)28-11-29-21(16)31/h11-13,17-19,23,34-35H,4-10H2,1-3H3,(H,26,32)(H,27,36)(H,40,41)(H,42,43)(H2,25,28,29)(H2,37,38,39)/t13-,17-,18-,19+,23-/m1/s1
InChI KeyQAQREVBBADEHPA-IEXPHMLFSA-N
Chemical Taxonomy
Description belongs to the class of organic compounds known as acyl coas. These are organic compounds containing a coenzyme A substructure linked to an acyl chain.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acyl thioesters
Direct ParentAcyl CoAs
Alternative Parents
Substituents
  • Coenzyme a or derivatives
  • Purine ribonucleoside 3',5'-bisphosphate
  • Purine ribonucleoside bisphosphate
  • Purine ribonucleoside diphosphate
  • Ribonucleoside 3'-phosphate
  • Pentose phosphate
  • Pentose-5-phosphate
  • Glycosyl compound
  • N-glycosyl compound
  • 6-aminopurine
  • Monosaccharide phosphate
  • Organic pyrophosphate
  • Pentose monosaccharide
  • Imidazopyrimidine
  • Purine
  • Aminopyrimidine
  • Monoalkyl phosphate
  • Alkyl phosphate
  • Pyrimidine
  • Phosphoric acid ester
  • Imidolactam
  • Monosaccharide
  • N-substituted imidazole
  • Organic phosphoric acid derivative
  • Tetrahydrofuran
  • Azole
  • Imidazole
  • Heteroaromatic compound
  • Thiocarboxylic acid ester
  • Secondary alcohol
  • Amino acid or derivatives
  • Carbothioic s-ester
  • Thiocarboxylic acid or derivatives
  • Sulfenyl compound
  • Propargyl-type 1,3-dipolar organic compound
  • Carboximidic acid
  • Carboximidic acid derivative
  • Carboxylic acid derivative
  • Organic 1,3-dipolar compound
  • Organoheterocyclic compound
  • Azacycle
  • Oxacycle
  • Organopnictogen compound
  • Organic oxygen compound
  • Organic oxide
  • Amine
  • Hydrocarbon derivative
  • Primary amine
  • Carbonyl group
  • Alcohol
  • Organosulfur compound
  • Organic nitrogen compound
  • Organonitrogen compound
  • Organooxygen compound
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic heteropolycyclic compounds
External Descriptors
Ontology
Physiological effect

Health effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Biological role:

Industrial application:

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 Solubility4.27 g/LALOGPS
logP-0.31ALOGPS
logP-5.2ChemAxon
logS-2.3ALOGPS
pKa (Strongest Acidic)0.82ChemAxon
pKa (Strongest Basic)4.01ChemAxon
Physiological Charge-4ChemAxon
Hydrogen Acceptor Count17ChemAxon
Hydrogen Donor Count9ChemAxon
Polar Surface Area363.63 ŲChemAxon
Rotatable Bond Count21ChemAxon
Refractivity176.83 m³·mol⁻¹ChemAxon
Polarizability74.37 ųChemAxon
Number of Rings3ChemAxon
BioavailabilityNoChemAxon
Rule of FiveNoChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-000i-1901000220-8d8b91979e9c40176540Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-000i-0912000000-7c91e2c2f2f0d7b4a3adSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-000i-2901000000-4311d5cc2b23055e1e3eSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0a7i-9830230550-4bec7e3f4052a0f39eefSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0560-4910100000-d7281a44fcd4120a6bcdSpectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-057i-6900100000-19dd2ec65951d55b39f7Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
  • Peroxisome
Biospecimen LocationsNot Available
Tissue LocationsNot Available
Pathways
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDDB02912
Phenol Explorer Compound IDNot Available
FooDB IDFDB022529
KNApSAcK IDNot Available
Chemspider ID83731
KEGG Compound IDC00100
BioCyc IDPROPIONYL-COA
BiGG IDNot Available
Wikipedia LinkPropionyl-CoA
METLIN IDNot Available
PubChem Compound92753
PDB IDNot Available
ChEBI ID15539
Food Biomarker OntologyNot Available
VMH IDNot Available
References
Synthesis ReferenceSokatch, John R.; Sanders, Lois E.; Marshall, Vincent P. Oxidation of methylmalonate semialdehyde to propionyl coenzyme A in Pseudomonas aeruginosa grown on valine. Journal of Biological Chemistry (1968), 243(10), 2500-6.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Winter SC, Buist NR: Cardiomyopathy in childhood, mitochondrial dysfunction, and the role of L-carnitine. Am Heart J. 2000 Feb;139(2 Pt 3):S63-9. [PubMed:10650319 ]

Only showing the first 10 proteins. There are 122 proteins in total.

Show all enzymes and transporters

Enzymes

Enzyme Details
1. Malonyl-CoA decarboxylase, mitochondrial
General function:
Involved in malonyl-CoA decarboxylase activity
Specific function:
Catalyzes the conversion of malonyl-CoA to acetyl-CoA. In the fatty acid biosynthesis MCD selectively removes malonyl-CoA and thus assures that methyl-malonyl-CoA is the only chain elongating substrate for fatty acid synthase and that fatty acids with multiple methyl side chains are produced. In peroxisomes it may be involved in degrading intraperoxisomal malonyl-CoA, which is generated by the peroxisomal beta-oxidation of odd chain-length dicarboxylic fatty acids.
Gene Name:
MLYCD
Uniprot ID:
O95822
Molecular weight:
55002.94
Enzyme Details
2. Carnitine O-acetyltransferase
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
Enzyme Details
3. 3-ketoacyl-CoA thiolase, mitochondrial
General function:
Involved in transferase activity, transferring acyl groups other than amino-acyl groups
Specific function:
Abolishes BNIP3-mediated apoptosis and mitochondrial damage.
Gene Name:
ACAA2
Uniprot ID:
P42765
Molecular weight:
41923.82
Reactions
Propionyl-CoA + Acetyl-CoA → Coenzyme A + 2-Methylacetoacetyl-CoAdetails
Propionyl-CoA + Chenodeoxycholoyl-CoA → Coenzyme A + 3a,7a-Dihydroxy-5b-cholestanoyl-CoAdetails
Enzyme Details
4. Acetyl-coenzyme A synthetase, cytoplasmic
General function:
Involved in acetate-CoA ligase activity
Specific function:
Activates acetate so that it can be used for lipid synthesis or for energy generation.
Gene Name:
ACSS2
Uniprot ID:
Q9NR19
Molecular weight:
78579.11
Reactions
Propinol adenylate + Coenzyme A → Adenosine monophosphate + Propionyl-CoAdetails
Enzyme Details
5. Acetyl-coenzyme A synthetase 2-like, mitochondrial
General function:
Involved in acetate-CoA ligase activity
Specific function:
Important for maintaining normal body temperature during fasting and for energy homeostasis. Essential for energy expenditure under ketogenic conditions (By similarity). Converts acetate to acetyl-CoA so that it can be used for oxidation through the tricarboxylic cycle to produce ATP and CO(2).
Gene Name:
ACSS1
Uniprot ID:
Q9NUB1
Molecular weight:
74625.88
Reactions
Propinol adenylate + Coenzyme A → Adenosine monophosphate + Propionyl-CoAdetails
Enzyme Details
6. 3-ketoacyl-CoA thiolase, peroxisomal
General function:
Involved in transferase activity, transferring acyl groups other than amino-acyl groups
Specific function:
Not Available
Gene Name:
ACAA1
Uniprot ID:
P09110
Molecular weight:
34664.46
Reactions
Propionyl-CoA + Acetyl-CoA → Coenzyme A + 2-Methylacetoacetyl-CoAdetails
Propionyl-CoA + Chenodeoxycholoyl-CoA → Coenzyme A + 3a,7a-Dihydroxy-5b-cholestanoyl-CoAdetails
Enzyme Details
7. Trifunctional enzyme subunit beta, mitochondrial
General function:
Involved in transferase activity, transferring acyl groups other than amino-acyl groups
Specific function:
Not Available
Gene Name:
HADHB
Uniprot ID:
P55084
Molecular weight:
51293.955
Reactions
Propionyl-CoA + Acetyl-CoA → Coenzyme A + 2-Methylacetoacetyl-CoAdetails
Propionyl-CoA + Chenodeoxycholoyl-CoA → Coenzyme A + 3a,7a-Dihydroxy-5b-cholestanoyl-CoAdetails
Enzyme Details
8. Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
General function:
Involved in oxidoreductase activity
Specific function:
Plays a role in valine and pyrimidine metabolism. Binds fatty acyl-CoA.
Gene Name:
ALDH6A1
Uniprot ID:
Q02252
Molecular weight:
57839.31
Reactions
2-Methyl-3-oxopropanoic acid + Coenzyme A + Water + NAD → Propionyl-CoA + Hydrogen carbonate + NADHdetails
(S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD → Propionyl-CoA + Carbon dioxide + NADH + Hydrogen Iondetails
Enzyme Details
9. Long-chain specific acyl-CoA dehydrogenase, mitochondrial
General function:
Involved in acyl-CoA dehydrogenase activity
Specific function:
Not Available
Gene Name:
ACADL
Uniprot ID:
P28330
Molecular weight:
47655.275
Enzyme Details
10. Short-chain specific acyl-CoA dehydrogenase, mitochondrial
General function:
Involved in acyl-CoA dehydrogenase activity
Specific function:
Not Available
Gene Name:
ACADS
Uniprot ID:
P16219
Molecular weight:
44296.705

Transporters

Enzyme Details
1. Long-chain fatty acid transport protein 1
General function:
Lipid transport and metabolism
Specific function:
Involved in translocation of long-chain fatty acids (LFCA) across the plasma membrane. The LFCA import appears to be hormone-regulated in a tissue-specific manner. In adipocytes, but not myocytes, insulin induces a rapid translocation of FATP1 from intracellular compartments to the plasma membrane, paralleled by increased LFCA uptake. May act directly as a bona fide transporter, or alternatively, in a cytoplasmic or membrane- associated multimeric protein complex to trap and draw fatty acids towards accumulation. Plays a pivotal role in regulating available LFCA substrates from exogenous sources in tissues undergoing high levels of beta-oxidation or triglyceride synthesis. May be involved in regulation of cholesterol metabolism. Has acyl-CoA ligase activity for long-chain and very-long-chain fatty acids
Gene Name:
SLC27A1
Uniprot ID:
Q6PCB7
Molecular weight:
71107.5

Only showing the first 10 proteins. There are 122 proteins in total.

Show all enzymes and transporters