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Bovine Metabolome Database



Showing metabocard for Alpha-ketoisovaleric acid (BMDB00019)

Legend: metabolite field enzyme field

Version 1.0
Creation Date 2005-11-16 15:48:42
Update Date 2009-07-07 12:51:32
Accession Number BMDB00019
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.
Synonyms
  1. 2-Ketoisovalerate
  2. 2-Ketoisovaleric acid
  3. 2-Oxo-3-methylbutanoate
  4. 2-Oxo-3-methylbutanoic acid
  5. 2-Oxo-3-methylbutyrate
  6. 2-Oxo-3-methylbutyric acid
  7. 2-Oxoisovalerate
  8. 2-Oxoisovaleric acid
  9. 2-keto-3-Methylbutyrate
  10. 2-keto-3-Methylbutyric acid
  11. 3-Methyl-2-oxobutanoate
  12. 3-Methyl-2-oxobutanoic acid
  13. 3-Methyl-2-oxobutyrate
  14. 3-Methyl-2-oxobutyric acid
  15. 3-methyl-2-oxo-Butanoate
  16. 3-methyl-2-oxo-Butanoic acid
  17. 3-methyl-2-oxo-Butyrate
  18. 3-methyl-2-oxo-Butyric acid
  19. Dimethylpyruvate
  20. Dimethylpyruvic acid
  21. Isopropylglyoxylate
  22. Isopropylglyoxylic acid
  23. Ketovaline
  24. a-Ketoisovalerate
  25. a-Oxo-b-methylbutyrate
  26. a-Oxo-b-methylbutyric acid
  27. a-Oxoisovalerate
  28. a-Oxoisovaleric acid
  29. a-keto-Isovalerate
  30. a-keto-Isovaleric acid
  31. a-keto-b-Methylbutyrate
  32. a-keto-b-Methylbutyric acid
  33. alpha-Ketoisovalerate
  34. alpha-Ketoisovaleric acid
  35. 3-Methyl-2-oxobutinoic acid
  36. alpha-Oxo-beta-methylbutyrate
  37. alpha-Oxo-beta-methylbutyric acid
  38. alpha-Oxoisovalerate
  39. alpha-Oxoisovaleric acid
  40. alpha-keto-Isovalerate
  41. alpha-keto-Isovaleric acid
  42. alpha-keto-beta-Methylbutyrate
  43. alpha-keto-beta-Methylbutyric acid
  44. a-ketoisovaleric acid
  45. 3-Methyl-2-oxobutinoate
Chemical IUPAC Name 3-methyl-2-oxo-butanoic acid
Chemical Formula C5H8O3
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Organic acids
Class
  • Keto-Acids
Sub Class
  • Short chain keto-acids
Family
  • Mammalian_Metabolite
Species
  • ketone; carboxylic acid
Biofunction
Application
Source
  • Endogenous
Average Molecular Weight 116.115
Monoisotopic Molecular Weight 116.047340
Isomeric SMILES CC(C)C(=O)C(O)=O
Canonical SMILES CC(C)C(=O)C(O)=O
KEGG Compound ID C00141 Link Image
BioCyc ID 2-KETO-ISOVALERATE Link Image
BiGG ID 34011 Link Image
Wikipedia Link Not Available
METLIN ID 5091 Link Image
PubChem Compound 49 Link Image
PubChem Substance 824662 Link Image
ChEBI ID 16530 Link Image
CAS Registry Number 759-05-7
InChI Identifier InChI=1/C5H8O3/c1-3(2)4(6)5(7)8/h3H,1-2H3,(H,7,8)
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.
Melting Point (Experimental) 31.5 oC
Experimental Water Solubility Not Available Source: PhysProp
Predicted Water Solubility 1000.0 mg/mL [MEYLAN,WM et al. (1996)]; 30.2 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge -1
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity 0.49 [Predicted by ALOGPS]; 0.1 [Predicted by PubChem via XLOGP]; -0.33 [MEYLAN,WM & HOWARD,PH (1995)] Calculated using ALOGPS
Material Safety Data Sheet (MSDS)
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID 1HJG Link Image
Experimental PDB File Show
Experimental PDB Structure
Experimental 1H NMR Spectrum Download Spectrum
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Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Download Spectrum
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Predicted 1H NMR Spectrum Show Image
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Predicted 13C NMR Spectrum Show Image
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Mass Spectrum
Low Energy
Download File
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Medium Energy
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High Energy
Download File
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Simplified TOCSY Spectrum Show Image
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BMRB Spectrum Show Image
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Cellular Location
  • Cytoplasm
  • mitochondria
Biofluid Location Not Available
Tissue Location
Tissue References
Most Tissues
Concentrations (Normal) Not Available
Concentrations (Abnormal) Not Available
Pathway Names
  • Pantothenate and CoA Biosynthesis
  • Valine, Leucine and Isoleucine Degradation
HMDB Pathways
Name Pantothenate and CoA Biosynthesis
Image Show Link Image
Name Valine, Leucine and Isoleucine Degradation
Image Show Link Image
KEGG Pathways
Name Pantothenate and CoA Biosynthesis
Image Show Link Image
Name Valine, Leucine and Isoleucine Degradation
Image Show Link Image
SimCell Pathways
Name Valine, Leucine and Isoleucine Degradation
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Graph Show Link Image
SBML Download (XML) Link Image
General References Not Available
Metabolic Enzymes
  1. 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
  2. 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Enzyme 1 [top]
Enzyme 1 ID 637
Enzyme 1 Name 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Enzyme 1 Synonyms
  1. Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain
  2. BCKDH E1-alpha
Enzyme 1 Gene Name BCKDHA
Enzyme 1 Protein Sequence >2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
MQGSAKMAMAVAVAVARVWRPSRGLGRTGLPLLRLLGARGLARFHPHRWQQQQHFSSLDD
KPQFPGASAEFIDKLEFIQPNVISGIPIYRVMDRQGQIINPSEDPHLPQEKVLKFYKSMT
LLNTMDRILYESQRQGRISFYMTNYGEEGTHVGSAAALDDTDLVFGQYREAGVLMYRDYP
LELFMAQCYGNVSDLGKGRQMPVHYGCRERHFVTISSPLATQIPQAVGAAYAAKRANANR
VVICYFGEGAASEGDAHAGFNFAATLECPIIFFCRNNGYAISTPTSEQYRGDGIAARGPG
YGILSIRVDGNDVFAVYNATKEARRRAVAENQPFLIEAMTYRIGHHSTSDDSSAYRSVDE
VNYWDKQDHPISRLRHHLQSRGWWDDEQEKAWRKQSRKKVMEAFEQAERKLKPNPSLIFS
DVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFEK
Enzyme 1 Number of Residues 455
Enzyme 1 Molecular Weight 51678.0
Enzyme 1 Theoretical pI 8.61
Enzyme 1 GO Classification
Function
Process
Component
Enzyme 1 General Function Energy production and conversion
Enzyme 1 Specific Function The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components:branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3)
Enzyme 1 Pathways Not Available
Enzyme 1 Reactions Not Available
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • None
Enzyme 1 Transmembrane Regions
  • None
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 163239 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID P11178 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name ODBA_BOVIN Link Image
Enzyme 1 PDB ID 1U5B Link Image
Enzyme 1 PDB File Show
Enzyme 1 3D Structure
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence >1368 bp
TTTTTTGGGTTGGTTAGATTTCATGCAGGGGTCAGCCAAGATGGCGATGGCGGTGGCGGT
TGCGGTGGCCAGGGTTTGGAGACCAAGTCGAGGCTTGGGACGGACGGGCCTCCCGCTCCT
GCGGCTGCTTGGGGCTCGTGGGCTGGCTAGATTTCACCCCCACAGGTGGCAGCAACAGCA
GCACTTCTCGTCCCTGGATGACAAGCCGCAGTTCCCAGGGGCCTCAGCGGAGTTCATAGA
CAAGCTCGAATTCATCCAGCCCAATGTCATCTCTGGGATCCCCATCTACCGGGTCATGGA
CCGGCAGGGCCAGATCATCAACCCCAGCGAGGATCCCCACCTGCCCCAGGAGAAGGTGCT
CAAATTCTACAAGAGCATGACCCTGCTCAACACCATGGACCGCATCCTCTATGAATCCCA
GAGGCAGGGCCGCATATCCTTCTACATGACCAACTATGGCGAAGAGGGGACACACGTGGG
GAGCGCAGCAGCCCTGGACGACACAGACCTGGTGTTTGGCCAGTACCGGGAGGCAGGTGT
GCTCATGTACCGGGACTACCCGTTGGAGCTGTTCATGGCCCAGTGCTACGGCAACGTGAG
CGACCTGGGCAAGGGGCGCCAGATGCCCGTCCACTACGGCTGCAGGGAGCGTCACTTCGT
CACCATCTCCTCTCCACTGGCCACGCAGATCCCCCAGGCGGTCGGGGCAGCCTACGCGGC
CAAGAGGGCCAACGCTAACAGGGTGGTCATCTGTTACTTTGGAGAGGGGGCGGCCAGTGA
GGGGGACGCCCACGCCGGCTTCAACTTCGCCGCCACCCTCGAGTGCCCCATCATCTTCTT
CTGTCGGAACAACGGCTACGCCATCTCCACGCCCACCTCGGAGCAGTACCGCGGGGACGG
CATAGCGGCTCGAGGCCCCGGGTACGGCATCCTGTCCATCCGCGTGGACGGCAATGATGT
GTTTGCCGTGTACAACGCCACCAAGGAGGCCCGGCGGCGGGCCGTGGCGGAGAACCAGCC
CTTCCTCATTGAGGCCATGACCTACAGGATCGGGCACCACAGCACCAGTGACGACAGCTC
GGCGTACCGCTCAGTGGACGAGGTCAACTACTGGGACAAGCAGGACCACCCCATCTCCCG
GCTGCGGCATCACCTGCAGAGCCGCGGCTGGTGGGACGACGAGCAGGAGAAGGCCTGGAG
GAAGCAGTCCCGCAAGAAGGTAATGGAGGCCTTTGAGCAGGCTGAGCGGAAGCTGAAGCC
CAACCCCAGCTTGATCTTCTCGGACGTGTATCAGGAGATGCCTGCCCAGCTCCGCAAGCA
GCAGGAGTCTCTGGCACGTCACCTCCAGACCTATGGTGAACACTACCC
Enzyme 1 GenBank Gene ID J03759 Link Image
Enzyme 1 GeneCard ID BCKDHA Link Image
Enzyme 1 GenAtlas ID Not Available
Enzyme 1 HGNC ID Not Available
Enzyme 1 Chromosome Location Chromosome:1
Enzyme 1 Locus 19q13.1-q13.2
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References
  1. [PubMed Link Image]
Enzyme 1 Metabolite References Not Available
Enzyme 2 [top]
Enzyme 2 ID 638
Enzyme 2 Name 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Enzyme 2 Synonyms
  1. Branched-chain alpha-keto acid dehydrogenase E1 component beta chain
  2. BCKDH E1-beta
Enzyme 2 Gene Name BCKDHB
Enzyme 2 Protein Sequence >2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
MAAVAAFAGWLLRLRAAGADGPWRRLCGAGLSRGFLQSASAYGAAAQRRQVAHFTFQPDP
EPVEYGQTQKMNLFQAVTSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVF
NTPLCEQGIVGFGIGIAVTGATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSL
TIRSPWGCVGHGALYHSQSPEAFFAHCPGIKVVVPRSPFQAKGLLLSCIEDKNPCIFFEP
KILYRAAVEQVPVEPYNIPLSQAEVIQEGSDVTLVAWGTQVHVIREVAAMAQEKLGVSCE
VIDLRTILPWDVDTVCKSVIKTGRLLVSHEAPLTGGFASEISSTVQEECFLNLEAPISRV
CGYDTPFPHIFEPFYIPDKWKCYDALRKMINY
Enzyme 2 Number of Residues 392
Enzyme 2 Molecular Weight 42934.9
Enzyme 2 Theoretical pI 6.17
Enzyme 2 GO Classification
Function
Process
Component
Enzyme 2 General Function Energy production and conversion
Enzyme 2 Specific Function The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components:branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3)
Enzyme 2 Pathways Not Available
Enzyme 2 Reactions Not Available
Enzyme 2 Pfam Domain Function
Enzyme 2 Signals
  • None
Enzyme 2 Transmembrane Regions
  • None
Enzyme 2 Essentiality Not Available
Enzyme 2 GenBank ID Protein 506803 Link Image
Enzyme 2 UniProtKB/Swiss-Prot ID P21839 Link Image
Enzyme 2 UniProtKB/Swiss-Prot Entry Name ODBB_BOVIN Link Image
Enzyme 2 PDB ID 1X80 Link Image
Enzyme 2 PDB File Show
Enzyme 2 3D Structure
Enzyme 2 Cellular Location Not Available
Enzyme 2 Gene Sequence >1179 bp
ATGGCGGCTGTGGCGGCGTTCGCGGGCTGGCTGCTGCGGCTCCGTGCAGCCGGGGCCGAC
GGACCCTGGCGTCGGCTGTGTGGCGCGGGGCTGTCGAGGGGCTTCCTGCAGTCCGCCTCG
GCCTACGGGGCTGCGGCCCAGAGGCGGCAGGTGGCTCACTTCACTTTCCAGCCTGACCCG
GAGCCCGTGGAGTACGGGCAGACTCAGAAAATGAATCTCTTCCAGGCAGTAACAAGTGCC
TTAGATAACTCATTGGCCAAAGATCCTACGGCAGTAATATTTGGTGAAGACGTTGCCTTT
GGTGGAGTCTTTAGATGTACTGTCGGCTTGCGAGACAAGTATGGTAAAGATAGAGTTTTT
AATACCCCACTGTGTGAACAAGGAATCGTTGGATTTGGAATTGGAATCGCAGTCACCGGT
GCTACTGCCATAGCAGAAATTCAGTTTGCAGATTATATTTTCCCTGCTTTTGATCAGATT
GTTAATGAAGCTGCCAAGTATCGCTACCGGTCTGGGGACCTTTTTAATTGTGGAAGCCTC
ACCATCCGGTCCCCTTGGGGCTGTGTCGGCCACGGGGCTCTCTATCATTCCCAGAGTCCT
GAAGCTTTCTTTGCCCACTGCCCAGGAATCAAGGTGGTTGTACCCAGAAGCCCTTTCCAG
GCCAAGGGACTTCTTTTATCATGCATAGAGGATAAAAATCCTTGTATATTTTTTGAACCT
AAAATACTTTACAGGGCAGCAGTGGAGCAGGTTCCTGTAGAGCCATACAACATCCCCCTT
TCCCAAGCTGAAGTCATCCAAGAAGGGAGTGATGTCACTCTAGTTGCCTGGGGCACTCAG
GTTCATGTGATCCGAGAGGTGGATGCCATGGCTCAAGAGAAGCTTGGGGTGTCTTGTGAG
GTCATTGATCTGAGGACTATACTACCTTGGGATGTGGATACAGTTTGCAAGTCTGTGATC
AAAACAGGGCGACTGCTAGTAAGTCATGAGGCTCCTTTGACGGGCGGCTTTGCCTCTGAG
ATCAGCTCAACGGTTCAGGAAGAATGTTTCCTGAACCTGGAAGCTCCTATATCAAGGGTG
TGTGGGTACGATACACCGTTCCCTCACATTTTTGAACCGTTCTACATCCCAGACAAGTGG
AAGTGCTATGATGCCCTTCGAAAAATGATCAACTATTGA
Enzyme 2 GenBank Gene ID M33323 Link Image
Enzyme 2 GeneCard ID BCKDHB Link Image
Enzyme 2 GenAtlas ID Not Available
Enzyme 2 HGNC ID Not Available
Enzyme 2 Chromosome Location Chromosome:6
Enzyme 2 Locus 6q13-q15
Enzyme 2 SNPs SNPJam Report Link Image
Enzyme 2 General References
  1. [PubMed Link Image]
Enzyme 2 Metabolite References Not Available