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Record Information
Version4.0
StatusExpected but not Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-12-20 20:32:57 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
Thumb
Synonyms
ValueSource
2-Methylacetyl-CoAHMDB
2-Methylacetyl-coenzyme AHMDB
alpha-Methylacetyl-CoAHMDB
alpha-Methylacetyl-coenzyme AHMDB
Propanoyl-CoAHMDB
Propanoyl-coenzyme AHMDB
Propionyl-coenzyme AHMDB
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({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 Name[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[({hydroxy[hydroxy(3-hydroxy-2,2-dimethyl-3-[(2-{[2-(propanoylsulfanyl)ethyl]carbamoyl}ethyl)carbamoyl]propoxy)phosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid
CAS Registry Number317-66-8
SMILES
CCC(=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/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-UXYNFSPESA-N
Chemical Taxonomy
DescriptionThis compound 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
  • Beta amino acid or derivatives
  • Glycosyl compound
  • N-glycosyl compound
  • 6-aminopurine
  • Monosaccharide phosphate
  • Organic pyrophosphate
  • Pentose monosaccharide
  • Imidazopyrimidine
  • Purine
  • Monoalkyl phosphate
  • Aminopyrimidine
  • Imidolactam
  • N-acyl-amine
  • N-substituted imidazole
  • Organic phosphoric acid derivative
  • Monosaccharide
  • Pyrimidine
  • Alkyl phosphate
  • Fatty amide
  • Phosphoric acid ester
  • Tetrahydrofuran
  • Imidazole
  • Azole
  • Heteroaromatic compound
  • Carbothioic s-ester
  • Secondary alcohol
  • Thiocarboxylic acid ester
  • Carboxamide group
  • Secondary carboxylic acid amide
  • Amino acid or derivatives
  • Sulfenyl compound
  • Thiocarboxylic acid or derivatives
  • Organoheterocyclic compound
  • Azacycle
  • Oxacycle
  • Carboxylic acid derivative
  • Organosulfur compound
  • Organic oxygen compound
  • Hydrocarbon derivative
  • Carbonyl group
  • Organic nitrogen compound
  • Primary amine
  • Organopnictogen compound
  • Organic oxide
  • Organooxygen compound
  • Organonitrogen compound
  • Alcohol
  • Amine
  • Aromatic heteropolycyclic compound
Molecular FrameworkAromatic heteropolycyclic compounds
External DescriptorsNot Available
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-6.3ChemAxon
logS-2.3ALOGPS
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 Count21ChemAxon
Refractivity176.83 m³·mol⁻¹ChemAxon
Polarizability72.51 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-1921000110-a902ba2e846c5fa3a6f6View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0019-1911000000-73f7c171e662ee7ccd1dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0019-1910000000-e82bdc99f5bada8c440eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0a7i-9830232560-d45384d1d1a3fe0dd614View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-001i-4910200000-2a67602fe8e4a74a6abeView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-056r-8900100000-d3e8a1b9393d73c218b4View in MoNA
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 IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB022529
KNApSAcK IDNot Available
Chemspider ID388310
KEGG Compound IDC00100
BioCyc IDPROPIONYL-COA
BiGG ID33852
Wikipedia LinkNot Available
METLIN ID6130
PubChem Compound439164
PDB IDNot Available
ChEBI ID15539
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.

Enzymes

General function:
Involved in acyl-CoA dehydrogenase activity
Specific function:
This enzyme is specific for acyl chain lengths of 4 to 16.
Gene Name:
ACADM
Uniprot ID:
P11310
Molecular weight:
46587.98
Reactions
Propionyl-CoA + FAD → FADH + Acrylyl-CoAdetails
Electron-transferring flavoprotein + Propionyl-CoA → Reduced electron-transferring flavoprotein + Acrylyl-CoAdetails
General function:
Involved in acetate-CoA ligase activity
Specific function:
Not Available
Gene Name:
ACSS2
Uniprot ID:
Q96FY7
Molecular weight:
59346.4
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 + Carbonic acid + NADHdetails
(S)-Methylmalonic acid semialdehyde + Coenzyme A + NAD → Propionyl-CoA + Carbon dioxide + NADH + Hydrogen Iondetails
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 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
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
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
General function:
Involved in ligase activity
Specific function:
Not Available
Gene Name:
PCCB
Uniprot ID:
P05166
Molecular weight:
58215.13
Reactions
Adenosine triphosphate + Propionyl-CoA + Carbonic acid → ADP + Phosphoric acid + S-Methylmalonyl-CoAdetails
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. Plays an important role in fatty acid metabolism in brain and the acyl-CoAs produced may be utilized exclusively for the synthesis of the brain lipid.
Gene Name:
ACSL6
Uniprot ID:
Q9UKU0
Molecular weight:
80609.765
General function:
Involved in acyl-CoA dehydrogenase activity
Specific function:
Oxidizes the CoA esters of the bile acid intermediates di- and tri-hydroxycholestanoic acids.
Gene Name:
ACOX2
Uniprot ID:
Q99424
Molecular weight:
76826.14

Transporters

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.