| Record Information |
| Version |
3.5 |
| Creation Date |
2005-11-16 08:48:42 -0700 |
| Update Date |
2013-02-08 17:07:45 -0700 |
| HMDB ID |
HMDB00019 |
| Secondary Accession Numbers |
|
| Metabolite Identification |
| Common Name |
Alpha-ketoisovaleric acid |
| Description |
Alpha-ketoisovaleric acid is a branched chain organic acid which is a precursor to leucine and valine synthesis. It is also a degradation product from valine. The enzyme dihydroxy-acid dehydratase catalyzes the fourth step in the biosynthesis of isoleucine and valine, through the dehydration of 2, 3-dihydroxy-isovaleic acid into alpha-ketoisovaleric acid. |
| Structure |
Download:
MOL |
SDF |
SMILES |
InChI
Display:
2D Structure |
3D Structure
|
| Synonyms |
- 2-Keto-3-Methylbutyrate
- 2-Keto-3-Methylbutyric acid
- 2-Ketoisovalerate
- 2-Ketoisovaleric acid
- 2-Ketoisvaleric acid
- 2-Oxo-3-methyl-butyrate
- 2-Oxo-3-methylbutanoate
- 2-Oxo-3-methylbutanoic acid
- 2-Oxo-3-methylbutyrate
- 2-Oxo-3-methylbutyric acid
- 2-Oxoisovalerate
- 2-Oxoisovaleric acid
- 3-Methyl-2-oxo-Butanoate
- 3-Methyl-2-oxo-Butanoic acid
- 3-Methyl-2-oxo-Butyrate
- 3-Methyl-2-oxo-Butyric acid
- 3-Methyl-2-oxobutanoate
- 3-Methyl-2-oxobutanoic acid
- 3-Methyl-2-oxobutinoate
- 3-Methyl-2-oxobutinoic acid
- 3-Methyl-2-oxobutyrate
- 3-Methyl-2-oxobutyric acid
- a-Keto-b-Methylbutyrate
- a-Keto-b-Methylbutyric acid
- a-Keto-Isovalerate
- a-Keto-Isovaleric acid
- a-Ketoisovalerate
- a-Ketoisovaleric acid
- a-Oxo-b-methylbutyrate
- a-Oxo-b-methylbutyric acid
- a-Oxoisovalerate
- a-Oxoisovaleric acid
- alpha-Keto-beta-Methylbutyrate
- alpha-Keto-beta-Methylbutyric acid
- alpha-Keto-Isovalerate
- alpha-Keto-Isovaleric acid
- alpha-Ketoisovalerate
- alpha-Ketoisovaleric acid
- alpha-Oxo-beta-methylbutyrate
- alpha-Oxo-beta-methylbutyric acid
- alpha-Oxoisovalerate
- alpha-Oxoisovaleric acid
- Dimethylpyruvate
- Dimethylpyruvic acid
- Isopropylglyoxylate
- Isopropylglyoxylic acid
- Ketovaline
|
| Chemical Formula |
C5H8O3 |
| Average Molecular Weight |
116.1152 |
| Monoisotopic Molecular Weight |
116.047344122 |
| IUPAC Name |
3-methyl-2-oxobutanoic acid |
| Traditional IUPAC Name |
α-ketoisovalerate |
| CAS Registry Number |
759-05-7 |
| SMILES |
CC(C)C(=O)C(O)=O |
| InChI Identifier |
InChI=1S/C5H8O3/c1-3(2)4(6)5(7)8/h3H,1-2H3,(H,7,8) |
| InChI Key |
QHKABHOOEWYVLI-UHFFFAOYSA-N |
| Chemical Taxonomy |
| Kingdom |
Organic Compounds |
| Super Class |
Lipids |
| Class |
Fatty Acids and Conjugates |
| Sub Class |
Branched Fatty Acids |
| Other Descriptors |
- 2-oxo monocarboxylic acid(ChEBI)
- Aliphatic Acyclic Compounds
- Branched fatty acids(KEGG)
- Branched fatty acids(Lipidmaps)
- Organic Compounds
- short-chain fatty acid(ChEBI)
- straight-chain saturated fatty acid(ChEBI)
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| Substituents |
- Alpha Keto Acid
- Carboxylic Acid
- Ketone
- Short Chain Keto Acid
|
| Direct Parent |
Branched Fatty Acids |
| Ontology |
| Status |
Detected and Quantified |
| Origin |
|
| Biofunction |
- Cell signaling
- Fuel and energy storage
- Fuel or energy source
- Membrane integrity/stability
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| Application |
- Nutrients
- Stabilizers
- Surfactants and Emulsifiers
|
| Cellular locations |
- Cytoplasm
- Extracellular
- Membrane
- Mitochondria
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| Physical Properties |
| State |
Solid |
| Experimental Properties |
| Property |
Value |
Reference |
| Melting Point |
31.5 °C |
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 |
|
| Spectra |
|
| 1H NMR Spectrum |
| MS/MS Spectrum Quattro_QQQ 10 |
| MS/MS Spectrum Quattro_QQQ 25 |
| MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 10 |
| MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 20 |
| MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 30 |
| MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 40 |
| MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies ) |
| MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies ) |
| MS/MS Spectrum GC-MS |
| MS/MS Spectrum GC-MS |
| [1H,13C] 2D NMR Spectrum |
| [1H,1H] 2D NMR Spectrum |
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| Biological Properties |
| Cellular Locations |
- Cytoplasm
- Extracellular
- Membrane
- Mitochondria
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| Biofluid Locations |
- Blood
- Cerebrospinal Fluid (CSF)
- Urine
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| Tissue Location |
|
| Pathways |
| Name |
SMPDB Link |
KEGG Link |
| Valine, Leucine and Isoleucine Degradation |
SMP00032
|
map00280
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| Normal Concentrations |
|
| Blood |
Detected and Quantified |
|
14.0 (0.0-28.0) uM |
Children (1-13 year old) |
Both |
Normal |
Not Available |
| Blood |
Detected and Quantified |
|
11.0 +/- 1.7 uM |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Cerebrospinal Fluid (CSF) |
Detected and Quantified |
|
6 +/- 3 uM |
Not Specified |
Both |
Normal |
Not Available |
| Cerebrospinal Fluid (CSF) |
Detected and Quantified |
|
8.2 +/- 6.8 uM |
Adult (>18 years old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
0.16 +/- 0.48 umol/mmol creatinine |
Infant (0-1 year old) |
Both |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
0.5 (0.2-1.1) umol/mmol creatinine |
Adult (>18 years old) |
Both |
Normal |
urine by NMR
|
| Urine |
Detected and Quantified |
|
0.13 (0.00-0.54) umol/mmol creatinine |
Adult (>18 years old) |
Male |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
0.07 (0.01-0.45) umol/mmol creatinine |
Adult (>18 years old) |
Female |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
0.07 (0.01-0.45) umol/mmol creatinine |
Adult (>18 years old) |
Female |
Normal |
Not Available |
| Urine |
Detected and Quantified |
|
1.00 (0.00-2.00) umol/mmol creatinine |
Adult (>18 years old) |
Both |
Normal |
Not Available |
|
| Abnormal Concentrations |
|
| Blood |
Detected and Quantified |
|
10.7 +/- 5.5 uM |
Adult (>18 years old) |
Not Specified |
Heart Transplant |
Not Available |
| Urine |
Detected and Quantified |
|
550.00 (300.00-800.00) umol/mmol creatinine |
Adult (>18 years old) |
Both |
Maple syrup urine disease |
Not Available |
|
| Associated Disorders and Diseases |
| Disease References |
| Maple syrup urine disease |
- http://www.metagene.de/program/d.prg?mp=MAPLE%20SYRUP%20URINE%20DISEASE
|
|
| Associated OMIM IDs |
- 248600
(Maple syrup urine disease)
|
| External Links |
| DrugBank ID |
DB04074  |
| Phenol Explorer Compound ID |
Not Available |
| Phenol Explorer Metabolite ID |
Not Available |
| FoodDB ID |
FDB012250 |
| KNApSAcK ID |
C00007623  |
| Chemspider ID |
48  |
| KEGG Compound ID |
C00141  |
| BioCyc ID |
2-KETO-ISOVALERATE  |
| BiGG ID |
34011  |
| Wikipedia Link |
Not Available |
| NuGOwiki Link |
HMDB00019  |
| Metagene Link |
HMDB00019  |
| METLIN ID |
5091  |
| PubChem Compound |
49  |
| PDB ID |
KIV  |
| ChEBI ID |
16530  |
| References |
| Synthesis Reference |
Pirrung, Michael C.; Ha, Hyun Joon; Holmes, Christopher P. Purification and inhibition of spinach a,b-dihydroxyacid dehydratase . Journal of Organic Chemistry (1989), 54(7), 1543-8. |
| Material Safety Data Sheet (MSDS) |
Download (PDF)
|
| General References |
- Schaefer K, von Herrath D, Erley CM, Asmus G: Calcium ketovaline as new therapy for uremic hyperphosphatemia. Miner Electrolyte Metab. 1990;16(6):362-4.
Pubmed: 2089249
- Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38.
Pubmed: 2026685
- Livesey G, Lund P: Binding of branched-chain 2-oxo acids to bovine serum albumin. Biochem J. 1982 Apr 15;204(1):265-72.
Pubmed: 7115325
- Shigematsu Y, Kikuchi K, Momoi T, Sudo M, Kikawa Y, Nosaka K, Kuriyama M, Haruki S, Sanada K, Hamano N, et al.: Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease. J Inherit Metab Dis. 1983;6(4):183-9.
Pubmed: 6422161
- Chuang DT, Niu WL, Cox RP: Activities of branched-chain 2-oxo acid dehydrogenase and its components in skin fibroblasts from normal and classical-maple-syrup-urine-disease subjects. Biochem J. 1981 Oct 15;200(1):59-67.
Pubmed: 6895847
- Lee SH, Kim SO, Chung BC: Gas chromatographic-mass spectrometric determination of urinary oxoacids using O-(2,3,4,5,6-pentafluorobenzyl)oxime-trimethylsilyl ester derivatization and cation-exchange chromatography. J Chromatogr B Biomed Sci Appl. 1998 Nov 20;719(1-2):1-7.
Pubmed: 9869358
- Gallina DL, Dominguez JM, Hoschoian JC, Barrio JR: Maintenance of nitrogen balance in a young woman by substitution of -ketoisovaleric acid for valine. J Nutr. 1971 Sep;101(9):1165-7.
Pubmed: 5096137
- Schauder P, Schroder K, Langenbeck U: Serum branched-chain amino and keto acid response to a protein-rich meal in man. Ann Nutr Metab. 1984;28(6):350-6.
Pubmed: 6393856
- Tsuchiya H, Hashizume I, Tokunaga T, Tatsumi M, Takagi N, Hayashi T: High-performance liquid chromatography of alpha-keto acids in human saliva. Arch Oral Biol. 1983;28(11):989-92.
Pubmed: 6581765
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| Enzymes |
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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 |
|
| Name: |
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
|
| 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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