Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2013-04-22 22:23:29 UTC
HMDB IDHMDB01041
Secondary Accession Numbers
  • HMDB02021
  • HMDB02191
  • HMDB02253
Metabolite Identification
Common Name2-Methylbutyryl-CoA
Description2-Methylbutyryl-CoA is a a product of isoleucine catabolism. It is converted to Tiglyl-CoA by short/branched-chain acyl-CoA dehydrogenase. 2-Methylbutyryl-CoA dehydrogenase deficiency, also called 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency or MBHD, is an inherited disorder in which the body is unable to process the amino acid isoleucine properly. It is caused by a mutation in the HADH2 gene. Untreated MBHD can lead to progressive loss of motor skills, to mental retardation and to epilepsy. 2-Methylbutyryl-CoA is a substrate for Acyl-CoA dehydrogenase (short-chain specific, mitochondrial), Acyl-CoA dehydrogenase (medium-chain specific, mitochondrial) and Acyl-CoA dehydrogenase (long-chain specific, mitochondrial).
Structure
Thumb
Synonyms
  1. (S)-2-methylbutyryl-CoA
  2. (S)-2-methylbutyryl-Coenzyme A
  3. 2-Methylbutanoyl-CoA
  4. 2-Methylbutanoyl-Coenzyme A
  5. 2-Methylbutyryl-CoA
  6. 2-Methylbutyryl-Coenzyme A
  7. 3'-Phosphoadenosine 5'-{3-[(3R)-3-hydroxy-2,2-dimethyl-4-{[3-({2-[(2-methylbutanoyl)sulfanyl]ethyl}amino)-3-oxopropyl]amino}-4-oxobutyl] dihydrogen diphosphate}
  8. a-Methylbutyryl-CoA
  9. alpha-Methylbutyryl-CoA
  10. alpha-Methylbutyryl-Coenzyme A
  11. Methylbutanoyl-CoA
  12. Methylbutanoyl-Coenzyme A
Chemical FormulaC26H44N7O17P3S
Average Molecular Weight851.651
Monoisotopic Molecular Weight851.172723243
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-[(2-methylbutanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy)phosphoryl]oxy})phosphoryl]oxy}methyl)oxolan-3-yl]oxy}phosphonic acid
Traditional IUPAC Name[(2R,3S,4R,5R)-5-(6-aminopurin-9-yl)-4-hydroxy-2-[({hydroxy[hydroxy(3-hydroxy-2,2-dimethyl-3-{[2-({2-[(2-methylbutanoyl)sulfanyl]ethyl}carbamoyl)ethyl]carbamoyl}propoxy)phosphoryl]oxyphosphoryl}oxy)methyl]oxolan-3-yl]oxyphosphonic acid
CAS Registry Number6712-02-3
SMILES
CCC(C)C(=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/C26H44N7O17P3S/c1-5-14(2)25(38)54-9-8-28-16(34)6-7-29-23(37)20(36)26(3,4)11-47-53(44,45)50-52(42,43)46-10-15-19(49-51(39,40)41)18(35)24(48-15)33-13-32-17-21(27)30-12-31-22(17)33/h12-15,18-20,24,35-36H,5-11H2,1-4H3,(H,28,34)(H,29,37)(H,42,43)(H,44,45)(H2,27,30,31)(H2,39,40,41)/t14?,15-,18-,19-,20?,24-/m1/s1
InChI KeyLYNVNYDEQMMNMZ-PCLZRLGGSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassLipids
ClassFatty Acid Esters
Sub ClassAcyl CoAs
Other Descriptors
  • Aromatic Heteropolycyclic Compounds
Substituents
  • 1 Phosphoribosyl Imidazole
  • Aminopyrimidine
  • Carboxamide Group
  • Carboxylic Thioester
  • Coenzyme A
  • Glycosyl Compound
  • Imidazole
  • Imidazopyrimidine
  • Monosaccharide Phosphate
  • N Glycosyl Compound
  • Organic Hypophosphite
  • Organic Phosphite
  • Organic Pyrophosphate
  • Oxolane
  • Pentose Monosaccharide
  • Phosphoric Acid Ester
  • Purine
  • Purine Ribonucleoside 3',5' Bisphosphate
  • Pyrimidine
  • Saccharide
  • Secondary Alcohol
  • Secondary Carboxylic Acid Amide
  • Thiocarboxylic Acid Ester
Direct ParentAcyl CoAs
Ontology
StatusExpected and Not Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Fuel and energy storage
  • Fuel or energy source
  • Lipid biosynthesis, Fatty acid transport
  • 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 solubility3.91 g/LALOGPS
logP-0.14ALOGPS
logP-5.3ChemAxon
logS-2.3ALOGPS
pKa (strongest acidic)0.83ChemAxon
pKa (strongest basic)4.95ChemAxon
physiological charge-4ChemAxon
hydrogen acceptor count17ChemAxon
hydrogen donor count9ChemAxon
polar surface area363.63ChemAxon
rotatable bond count22ChemAxon
refractivity186.01ChemAxon
polarizability76.46ChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Extracellular
  • Membrane
  • Mitochondria
Biofluid LocationsNot Available
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Valine, Leucine and Isoleucine DegradationSMP00032map00280
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 IDFDB022387
KNApSAcK IDNot Available
Chemspider ID388491
KEGG Compound IDC01033
BioCyc ID2-METHYL-BUTYRYL-COA
BiGG ID36663
Wikipedia LinkNot Available
NuGOwiki LinkHMDB01041
Metagene LinkHMDB01041
METLIN ID5960
PubChem Compound439371
PDB IDNot Available
ChEBI ID15477
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General ReferencesNot Available

Only showing the first 50 proteins. There are 112 proteins in total.

Enzymes

Gene Name:
ACAA1
Uniprot ID:
P09110
Gene Name:
ACOX3
Uniprot ID:
O15254
Gene Name:
LIPC
Uniprot ID:
P11150
Gene Name:
DGAT1
Uniprot ID:
O75907
Gene Name:
SCD
Uniprot ID:
O00767
Gene Name:
SOAT2
Uniprot ID:
O75908
Gene Name:
SOAT1
Uniprot ID:
P35610
Gene Name:
ACSL4
Uniprot ID:
O60488
Gene Name:
ACSL1
Uniprot ID:
P33121
Gene Name:
ACSL6
Uniprot ID:
Q9UKU0
Gene Name:
ACSL5
Uniprot ID:
Q9ULC5
Gene Name:
ACSL3
Uniprot ID:
O95573
Gene Name:
ACOT4
Uniprot ID:
Q8N9L9
Gene Name:
ACOT8
Uniprot ID:
O14734
Gene Name:
ACADSB
Uniprot ID:
P45954
Reactions
2-Methylbutyryl-CoA + electron-transfer flavoprotein unknown (E)-2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + Hydrogen Iondetails
Gene Name:
SLC27A2
Uniprot ID:
O14975
Gene Name:
DBI
Uniprot ID:
P07108
Gene Name:
FABP3
Uniprot ID:
P05413
Gene Name:
SLC27A5
Uniprot ID:
Q9Y2P5
Gene Name:
MOGAT2
Uniprot ID:
Q3SYC2
Gene Name:
SLC27A3
Uniprot ID:
Q5K4L6
Gene Name:
ACAD10
Uniprot ID:
Q6JQN1

Transporters

Only showing the first 50 proteins. There are 112 proteins in total.