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Showing metabocard for Ketoleucine (HMDB0000695)

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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-05-30 20:55:52 UTC
HMDB IDHMDB0000695
Secondary Accession Numbers
  • HMDB0000762
  • HMDB00695
  • HMDB00762
Metabolite Identification
Common NameKetoleucine
DescriptionKetoleucine is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. Ketoleucine is both a neurotoxin and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of ketoleucine are associated with maple syrup urine disease (MSUD). MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. The symptoms of MSUD often show in infancy and lead to severe brain damage if untreated. MSUD may also present later depending on the severity of the disease. If left untreated in older individuals, during times of metabolic crisis, symptoms of the condition include uncharacteristically inappropriate, extreme, or erratic behaviour and moods, hallucinations, anorexia, weight loss, anemia, diarrhea, vomiting, dehydration, lethargy, oscillating hypertonia and hypotonia, ataxia, seizures, hypoglycemia, ketoacidosis, opisthotonus, pancreatitis, rapid neurological decline, and coma. In maple syrup urine disease, the brain concentration of branched-chain ketoacids can increase 10- to 20-fold. This leads to a depletion of glutamate and a consequent reduction in the concentration of brain glutamine, aspartate, alanine, and other amino acids. The result is a compromise of energy metabolism because of a failure of the malate-aspartate shuttle and a diminished rate of protein synthesis (PMID: 15930465 ).
Structure
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MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0000695 (Ketoleucine)

  Mrv1652309042000062D          

  9  8  0  0  0  0            999 V2000
10023.952810024.1004    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10024.669810024.5123    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10025.383310024.1004    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10026.098710024.5123    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10025.383310023.2754    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10023.238410024.5123    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10022.523910024.1004    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10023.238410025.3371    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10023.952810023.2754    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  6  1  0  0  0  0
  1  9  2  0  0  0  0
  2  3  1  0  0  0  0
  3  4  1  0  0  0  0
  3  5  1  0  0  0  0
  6  7  1  0  0  0  0
  6  8  2  0  0  0  0
M  END
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3D MOL for HMDB0000695 (Ketoleucine)

HMDB0000695
     RDKit          3D
Ketoleucine
 19 18  0  0  0  0  0  0  0  0999 V2000
   -2.0703    0.1757   -1.1606 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9527   -0.5142   -0.3787 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4345   -0.7518    1.0070 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.2662    0.3326   -0.4944 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.4513   -0.1546    0.1867 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.5124   -1.2279    0.8564 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.6774    0.6181    0.1184 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.6581    1.7008   -0.5434 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.8562    0.2187    0.7443 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0560    0.0073   -0.6943 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8598    1.2579   -1.2347 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1308   -0.2351   -2.1905 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8046   -1.4936   -0.8707 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5389   -0.9069    0.9590 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2770    0.1382    1.6560 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0344   -1.6846    1.4692 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0672    1.3723   -0.1625 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.5057    0.4166   -1.5876 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.1646    0.7304    1.5603 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  2  0
  5  7  1  0
  7  8  2  0
  7  9  1  0
  1 10  1  0
  1 11  1  0
  1 12  1  0
  2 13  1  0
  3 14  1  0
  3 15  1  0
  3 16  1  0
  4 17  1  0
  4 18  1  0
  9 19  1  0
M  END
Download

3D SDF for HMDB0000695 (Ketoleucine)

  Mrv1652309042000062D          

  9  8  0  0  0  0            999 V2000
10023.952810024.1004    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10024.669810024.5123    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10025.383310024.1004    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10026.098710024.5123    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10025.383310023.2754    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10023.238410024.5123    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10022.523910024.1004    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10023.238410025.3371    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10023.952810023.2754    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  6  1  0  0  0  0
  1  9  2  0  0  0  0
  2  3  1  0  0  0  0
  3  4  1  0  0  0  0
  3  5  1  0  0  0  0
  6  7  1  0  0  0  0
  6  8  2  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000695

> <DATABASE_NAME>
hmdb

> <SMILES>
CC(C)CC(=O)C(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C6H10O3/c1-4(2)3-5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)

> <INCHI_KEY>
BKAJNAXTPSGJCU-UHFFFAOYSA-N

> <FORMULA>
C6H10O3

> <MOLECULAR_WEIGHT>
130.1418

> <EXACT_MASS>
130.062994186

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
19

> <JCHEM_AVERAGE_POLARIZABILITY>
12.995994785498697

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
1

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
4-methyl-2-oxopentanoic acid

> <ALOGPS_LOGP>
0.82

> <JCHEM_LOGP>
1.4979975616666668

> <ALOGPS_LOGS>
-1.28

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA_STRONGEST_ACIDIC>
3.528051095084082

> <JCHEM_PKA_STRONGEST_BASIC>
-9.662090237480372

> <JCHEM_POLAR_SURFACE_AREA>
54.37

> <JCHEM_REFRACTIVITY>
31.7662

> <JCHEM_ROTATABLE_BOND_COUNT>
3

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
6.76e+00 g/l

> <JCHEM_TRADITIONAL_IUPAC>
ketoisocaproate

> <JCHEM_VEBER_RULE>
0

$$$$
Download

3D-SDF for HMDB0000695 (Ketoleucine)

HMDB0000695
     RDKit          3D
Ketoleucine
 19 18  0  0  0  0  0  0  0  0999 V2000
   -2.0703    0.1757   -1.1606 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9527   -0.5142   -0.3787 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4345   -0.7518    1.0070 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.2662    0.3326   -0.4944 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.4513   -0.1546    0.1867 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.5124   -1.2279    0.8564 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.6774    0.6181    0.1184 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.6581    1.7008   -0.5434 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.8562    0.2187    0.7443 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0560    0.0073   -0.6943 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8598    1.2579   -1.2347 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1308   -0.2351   -2.1905 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8046   -1.4936   -0.8707 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5389   -0.9069    0.9590 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2770    0.1382    1.6560 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0344   -1.6846    1.4692 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.0672    1.3723   -0.1625 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.5057    0.4166   -1.5876 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.1646    0.7304    1.5603 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  2  0
  5  7  1  0
  7  8  2  0
  7  9  1  0
  1 10  1  0
  1 11  1  0
  1 12  1  0
  2 13  1  0
  3 14  1  0
  3 15  1  0
  3 16  1  0
  4 17  1  0
  4 18  1  0
  9 19  1  0
M  END
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PDB for HMDB0000695 (Ketoleucine)

HEADER    PROTEIN                                 04-SEP-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   04-SEP-20         0                                  
HETATM    1  C   UNK     0    8711.3798711.654   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0    8712.7178712.423   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0    8714.0498711.654   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0    8715.3848712.423   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0    8714.0498710.114   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0    8710.0458712.423   0.000  0.00  0.00           C+0
HETATM    7  O   UNK     0    8708.7118711.654   0.000  0.00  0.00           O+0
HETATM    8  O   UNK     0    8710.0458713.963   0.000  0.00  0.00           O+0
HETATM    9  O   UNK     0    8711.3798710.114   0.000  0.00  0.00           O+0
CONECT    1    2    6    9                                                  
CONECT    2    1    3                                                       
CONECT    3    2    4    5                                                  
CONECT    4    3                                                            
CONECT    5    3                                                            
CONECT    6    1    7    8                                                  
CONECT    7    6                                                            
CONECT    8    6                                                            
CONECT    9    1                                                            
MASTER        0    0    0    0    0    0    0    0    9    0   16    0
END
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3D PDB for HMDB0000695 (Ketoleucine)

COMPND    HMDB0000695
HETATM    1  C1  UNL     1      -2.070   0.176  -1.161  1.00  0.00           C  
HETATM    2  C2  UNL     1      -0.953  -0.514  -0.379  1.00  0.00           C  
HETATM    3  C3  UNL     1      -1.435  -0.752   1.007  1.00  0.00           C  
HETATM    4  C4  UNL     1       0.266   0.333  -0.494  1.00  0.00           C  
HETATM    5  C5  UNL     1       1.451  -0.155   0.187  1.00  0.00           C  
HETATM    6  O1  UNL     1       1.512  -1.228   0.856  1.00  0.00           O  
HETATM    7  C6  UNL     1       2.677   0.618   0.118  1.00  0.00           C  
HETATM    8  O2  UNL     1       2.658   1.701  -0.543  1.00  0.00           O  
HETATM    9  O3  UNL     1       3.856   0.219   0.744  1.00  0.00           O  
HETATM   10  H1  UNL     1      -3.056   0.007  -0.694  1.00  0.00           H  
HETATM   11  H2  UNL     1      -1.860   1.258  -1.235  1.00  0.00           H  
HETATM   12  H3  UNL     1      -2.131  -0.235  -2.191  1.00  0.00           H  
HETATM   13  H4  UNL     1      -0.805  -1.494  -0.871  1.00  0.00           H  
HETATM   14  H5  UNL     1      -2.539  -0.907   0.959  1.00  0.00           H  
HETATM   15  H6  UNL     1      -1.277   0.138   1.656  1.00  0.00           H  
HETATM   16  H7  UNL     1      -1.034  -1.685   1.469  1.00  0.00           H  
HETATM   17  H8  UNL     1       0.067   1.372  -0.163  1.00  0.00           H  
HETATM   18  H9  UNL     1       0.506   0.417  -1.588  1.00  0.00           H  
HETATM   19  H10 UNL     1       4.165   0.730   1.560  1.00  0.00           H  
CONECT    1    2   10   11   12
CONECT    2    3    4   13
CONECT    3   14   15   16
CONECT    4    5   17   18
CONECT    5    6    6    7
CONECT    7    8    8    9
CONECT    9   19
END
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SMILES for HMDB0000695 (Ketoleucine)

CC(C)CC(=O)C(O)=O
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INCHI for HMDB0000695 (Ketoleucine)

InChI=1S/C6H10O3/c1-4(2)3-5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)
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View in JSmolView NMR AssignmentsView Stereo Labels
Synonyms
Chemical FormulaC6H10O3
Average Molecular Weight130.1418
Monoisotopic Molecular Weight130.062994186
IUPAC Name4-methyl-2-oxopentanoic acid
Traditional Nameketoisocaproate
CAS Registry Number816-66-0
SMILES
CC(C)CC(=O)C(O)=O
InChI Identifier
InChI=1S/C6H10O3/c1-4(2)3-5(7)6(8)9/h4H,3H2,1-2H3,(H,8,9)
InChI KeyBKAJNAXTPSGJCU-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassKeto acids and derivatives
Sub ClassShort-chain keto acids and derivatives
Direct ParentShort-chain keto acids and derivatives
Alternative Parents
Substituents
  • Branched fatty acid
  • Methyl-branched fatty acid
  • Short-chain keto acid
  • Alpha-keto acid
  • Fatty acyl
  • Alpha-hydroxy ketone
  • Ketone
  • Carboxylic acid derivative
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Carbonyl group
  • Hydrocarbon derivative
  • Organic oxygen compound
  • Organooxygen compound
  • Organic oxide
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Organoleptic effect
Disposition
Biological locationSource
Process
Naturally occurring process
Role
Biological roleIndustrial application
Physical Properties
StateLiquid
Experimental Molecular Properties
PropertyValueReference
Melting Point8 - 10 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility32 mg/mLNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Astarita_neg125.330932474
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Neuron
  • Placenta
  • Prostate
  • Skeletal Muscle
Pathways
Normal Concentrations
Abnormal Concentrations
Associated Disorders and Diseases
Disease References
Maple syrup urine disease
  1. Wendel, U., Becker, K., Przyrembel, H. et al. (1980). Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids. Eur J Pediatr (1980) 134: 57. https://doi.org/10.1007/BF00442404. Eur J Pediatr.
Branched-chain Keto Acid Dehydrogenase Kinase Deficiency
  1. Novarino G, El-Fishawy P, Kayserili H, Meguid NA, Scott EM, Schroth J, Silhavy JL, Kara M, Khalil RO, Ben-Omran T, Ercan-Sencicek AG, Hashish AF, Sanders SJ, Gupta AR, Hashem HS, Matern D, Gabriel S, Sweetman L, Rahimi Y, Harris RA, State MW, Gleeson JG: Mutations in BCKD-kinase lead to a potentially treatable form of autism with epilepsy. Science. 2012 Oct 19;338(6105):394-7. doi: 10.1126/science.1224631. Epub 2012 Sep 6. [PubMed:22956686 ]
Colorectal cancer
  1. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
  2. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
  3. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Gout
  1. Shao T, Shao L, Li H, Xie Z, He Z, Wen C: Combined Signature of the Fecal Microbiome and Metabolome in Patients with Gout. Front Microbiol. 2017 Feb 21;8:268. doi: 10.3389/fmicb.2017.00268. eCollection 2017. [PubMed:28270806 ]
Rheumatoid arthritis
  1. Tie-juan ShaoZhi-xing HeZhi-jun XieHai-chang LiMei-jiao WangCheng-ping Wen. Characterization of ankylosing spondylitis and rheumatoid arthritis using 1H NMR-based metabolomics of human fecal extracts. Metabolomics. April 2016, 12:70 [Link]
Attachment loss
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Missing teeth
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Periodontal Probing Depth
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Tooth Decay
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Associated OMIM IDs
DrugBank IDDB03229
Phenol Explorer Compound IDNot Available
FooDB IDFDB030510
KNApSAcK IDC00019677
Chemspider ID69
KEGG Compound IDC00233
BioCyc ID2K-4CH3-PENTANOATE
BiGG ID34334
Wikipedia LinkNot Available
METLIN ID5663
PubChem Compound70
PDB IDNot Available
ChEBI ID48430
Food Biomarker OntologyNot Available
VMH ID4MOP
MarkerDB IDMDB00000223
Good Scents IDNot Available
References
Synthesis ReferenceTabuchi, Takeshi; Abe, Matazo. Fermentative preparation of a-ketoisocaproic acid. Jpn. Tokkyo Koho (1970), 3 pp.
Material Safety Data Sheet (MSDS)Not Available
General References

Enzymes

Enzyme Details
1. 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
General function:
Involved in catalytic activity
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).
Gene Name:
BCKDHB
Uniprot ID:
P21953
Molecular weight:
43122.065
Reactions
Ketoleucine + Thiamine pyrophosphate → 3-Methyl-1-hydroxybutyl-ThPP + Carbon dioxidedetails
Enzyme Details
2. 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
General function:
Involved in oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
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).
Gene Name:
BCKDHA
Uniprot ID:
P12694
Molecular weight:
50470.58
Reactions
Ketoleucine + Thiamine pyrophosphate → 3-Methyl-1-hydroxybutyl-ThPP + Carbon dioxidedetails
Enzyme Details
3. Branched-chain-amino-acid aminotransferase, cytosolic
General function:
Involved in catalytic activity
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine.
Gene Name:
BCAT1
Uniprot ID:
P54687
Molecular weight:
38644.77
Reactions
Leucine + Oxoglutaric acid → Ketoleucine + Glutamic aciddetails
Enzyme Details
4. Branched-chain-amino-acid aminotransferase, mitochondrial
General function:
Involved in catalytic activity
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids.
Gene Name:
BCAT2
Uniprot ID:
O15382
Molecular weight:
33776.315
Reactions
Leucine + Oxoglutaric acid → Ketoleucine + Glutamic aciddetails