| Record Information |
|---|
| Version |
3.5 |
| Creation Date |
2005-11-16 08:48:42 -0700 |
| Update Date |
2013-04-22 16:23:29 -0600 |
| HMDB ID |
HMDB01041 |
| Secondary Accession Numbers |
- HMDB02021
- HMDB02191
- HMDB02253
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| Metabolite Identification |
|---|
| Common Name |
2-Methylbutyryl-CoA |
| Description |
2-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 |
Download:
MOL |
SDF |
SMILES |
InChI
Display:
2D Structure |
3D Structure
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| Synonyms |
- (S)-2-methylbutyryl-CoA
- (S)-2-methylbutyryl-Coenzyme A
- 2-Methylbutanoyl-CoA
- 2-Methylbutanoyl-Coenzyme A
- 2-Methylbutyryl-CoA
- 2-Methylbutyryl-Coenzyme A
- 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}
- a-Methylbutyryl-CoA
- alpha-Methylbutyryl-CoA
- alpha-Methylbutyryl-Coenzyme A
- Methylbutanoyl-CoA
- Methylbutanoyl-Coenzyme A
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| Chemical Formula |
C26H44N7O17P3S |
| Average Molecular Weight |
851.651 |
| Monoisotopic Molecular Weight |
851.172723243 |
| 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 Number |
6712-02-3 |
| 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 Key |
LYNVNYDEQMMNMZ-PCLZRLGGSA-N |
| Chemical Taxonomy |
|---|
| Kingdom |
Organic Compounds |
| Super Class |
Lipids |
| Class |
Fatty Acid Esters |
| Sub Class |
Acyl CoAs |
| Other Descriptors |
- Aromatic Heteropolycyclic Compounds
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| 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
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| Direct Parent |
Acyl CoAs |
| Ontology |
|---|
| Status |
Expected and Not Quantified |
| Origin |
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| Biofunction |
- Cell signaling
- Fuel and energy storage
- Fuel or energy source
- Lipid biosynthesis, Fatty acid transport
- Membrane integrity/stability
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| Application |
- Nutrients
- Stabilizers
- Surfactants and Emulsifiers
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| Cellular locations |
- Extracellular
- Membrane
- Mitochondria
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| Physical Properties |
|---|
| State |
Solid |
| Experimental Properties |
| Property |
Value |
Reference |
| Melting Point |
Not Available |
Not Available |
| Boiling Point |
Not Available |
Not Available |
| Water Solubility |
Not Available |
Not Available |
| LogP |
Not Available |
Not Available |
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| Predicted Properties |
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| Spectra |
|---|
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Not Available
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| Biological Properties |
|---|
| Cellular Locations |
- Extracellular
- Membrane
- Mitochondria
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| Biofluid Locations |
Not Available
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| Tissue Location |
Not Available
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| Pathways |
| Name |
SMPDB Link |
KEGG Link |
| Valine, Leucine and Isoleucine Degradation |
SMP00032
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map00280 
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| Normal Concentrations |
|---|
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Not Available |
| Abnormal Concentrations |
|---|
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Not Available |
| Associated Disorders and Diseases |
|---|
| Disease References |
None |
| Associated OMIM IDs |
None |
| External Links |
|---|
| DrugBank ID |
Not Available |
| Phenol Explorer Compound ID |
Not Available |
| Phenol Explorer Metabolite ID |
Not Available |
| FoodDB ID |
FDB022387 |
| KNApSAcK ID |
Not Available |
| Chemspider ID |
388491 |
| KEGG Compound ID |
C01033 |
| BioCyc ID |
2-METHYL-BUTYRYL-COA |
| BiGG ID |
36663 |
| Wikipedia Link |
Not Available |
| NuGOwiki Link |
HMDB01041 |
| Metagene Link |
HMDB01041 |
| METLIN ID |
5960 |
| PubChem Compound |
439371 |
| PDB ID |
Not Available |
| ChEBI ID |
15477 |
| References |
|---|
| Synthesis Reference |
Not Available |
| Material Safety Data Sheet (MSDS) |
Not Available
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| General References |
Not Available
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| Enzymes |
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| Name: |
Peroxisomal acyl-coenzyme A oxidase 2
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| Reactions: |
- (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoA + H2O + acceptor = (24R,25R)-3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestan-26- oyl-CoA + reduced acceptor [RN:R07374]
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| Gene Name: |
ACOX2 |
| Uniprot ID: |
Q99424 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
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| Reactions: |
- 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2 [RN:R01701]
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| Gene Name: |
BCKDHB |
| Uniprot ID: |
P21953 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
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| Reactions: |
- 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2 [RN:R01701]
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| Gene Name: |
BCKDHA |
| Uniprot ID: |
P12694 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Acyl-CoA desaturase
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| Reactions: |
- stearoyl-CoA + 2 ferrocytochrome b5 + O2 + 2 H+ = oleoyl-CoA + 2 ferricytochrome b5 + 2 H2O [RN:R02222]
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| Gene Name: |
SCD |
| Uniprot ID: |
O00767 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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| Name: |
Bile acyl-CoA synthetase
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| Reactions: |
- (1) ATP + cholate + CoA = AMP + diphosphate + choloyl-CoA [RN:R02794]
- (2) ATP + (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oate + CoA = AMP + diphosphate + (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA [RN:R04580]
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| Gene Name: |
SLC27A5 |
| Uniprot ID: |
Q9Y2P5 |
| Protein Sequence: |
FASTA |
| Gene Sequence: |
FASTA |
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