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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-05-20 19:01:16 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
Thumb
Synonyms
ValueSource
2-oxo-4-METHYLPENTANOIC ACIDChEBI
2-OxoisocaproateChEBI
4-Methyl-2-oxopentanoateChEBI
4-Methyl-2-oxovaleric acidChEBI
alpha-Ketoisocaproic acidChEBI
2-oxo-4-METHYLPENTANOateGenerator
2-Oxoisocaproic acidGenerator
4-Methyl-2-oxopentanoic acidGenerator
4-Methyl-2-oxovalerateGenerator
a-KetoisocaproateGenerator
a-Ketoisocaproic acidGenerator
alpha-KetoisocaproateGenerator
α-ketoisocaproateGenerator
α-ketoisocaproic acidGenerator
2-keto-4-MethylvalerateHMDB
2-keto-4-Methylvaleric acidHMDB
2-KetoisocaproateHMDB
2-Ketoisocaproic acidHMDB
2-oxo-4-MethylvalerateHMDB
2-oxo-4-Methylvaleric acidHMDB
2-OxoleucineHMDB
4-Methyl-2-oxo-valerateHMDB
4-Methyl-2-oxo-valeric acidHMDB
a-KetoisocapronateHMDB
a-Ketoisocapronic acidHMDB
a-OxoisocaproateHMDB
a-Oxoisocaproic acidHMDB
alpha-keto-IsocaproateHMDB
alpha-keto-Isocaproic acidHMDB
alpha-KetoisocapronateHMDB
alpha-Ketoisocapronic acidHMDB
alpha-OxoisocaproateHMDB
alpha-Oxoisocaproic acidHMDB
KetoisocaproateHMDB
Ketoisocaproic acidHMDB
MethyloxovalerateHMDB
Methyloxovaleric acidHMDB
OxoisocaproateHMDB
Oxoisocaproic acidHMDB
Keto-leucineHMDB
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
DescriptionThis compound 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
Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Indirect biological role:

Biological role:

Industrial application:

Physical Properties
StateLiquid
Experimental Properties
PropertyValueReference
Melting Point8 - 10 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility32 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility6.76 g/LALOGPS
logP0.82ALOGPS
logP1.5ChemAxon
logS-1.3ALOGPS
pKa (Strongest Acidic)3.53ChemAxon
pKa (Strongest Basic)-9.7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity31.77 m³·mol⁻¹ChemAxon
Polarizability13 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-000i-9400000000-49d055f04bfe1877ac5bView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)splash10-029j-8930000000-c6c54bf6ee2995a2195bView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0002-1910000000-0ba38257e25873312809View in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-0a5c-4930000000-61247369a805fa2a77a7View in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 1 TMS)splash10-000i-9510000000-8bfc5a54e29f65edf361View in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 1 TMS)splash10-00lj-9620000000-aa8409ceb2359d903f38View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-9400000000-49d055f04bfe1877ac5bView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-029j-8930000000-c6c54bf6ee2995a2195bView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0002-1910000000-0ba38257e25873312809View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0a5c-4930000000-61247369a805fa2a77a7View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-000i-9510000000-8bfc5a54e29f65edf361View in MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00lj-9620000000-aa8409ceb2359d903f38View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00ks-7930000000-4e9cfd715fa884d30e7fView in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-9500000000-709ebcb57f006703c269View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0006-9000000000-e410bce23706f826f8ddView in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-0006-9400000000-c4f06cea1e27a2b24660View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-0006-9000000000-bed7ac074a60210971e3View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-014i-9200000000-2fa08d2b4df406cf63eaView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-014j-9000000000-045be8e8dca3f93d45baView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-004i-0900000000-237fa8ad6bfb929be31eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-004r-9700000000-8d38d24fce78b1ff13e6View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-0a59-9000000000-759cfc0817917283d1a5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-0a4l-9000000000-2a068ada71fbe0a5a988View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0a4l-9000000000-58e61e31f3c96d84d799View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-004i-0900000000-237fa8ad6bfb929be31eView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-004r-9700000000-8d38d24fce78b1ff13e6View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0a59-9000000000-759cfc0817917283d1a5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0a4l-9000000000-2a068ada71fbe0a5a988View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0a4l-9000000000-58e61e31f3c96d84d799View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-ITFT , negativesplash10-004i-0900000000-556a0d1cf568609752d1View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT , negativesplash10-004r-5900000000-20f6923c8ed45e9fa088View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-08gr-7900000000-31021fda89eb6535185fView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0btm-9200000000-3bf5df4b557ef87267f4View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a4i-9000000000-37839bf70647d24b94a4View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-004i-4900000000-796467909d1d4c087a07View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-01ri-9400000000-0abcd021721f32c10e42View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-015l-9000000000-5c33a47dfeb09a182983View in MoNA
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR1H NMR SpectrumNot AvailableView in JSpectraViewer
1D NMR13C NMR SpectrumNot AvailableView in JSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableView in JSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Muscle
  • Neuron
  • Prostate
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified28.0 (0.0-58.0) uMAdult (>18 years old)BothNormal details
BloodDetected but not Quantified Adult (>18 years old)Female
Normal
details
BloodDetected and Quantified33.5 +/- 8.2 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected and Quantified25-28 uMAdolescent (13-18 years old)FemaleNormal details
BloodDetected and Quantified18 uMAdult (>18 years old)FemaleNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified5.0 +/- 4.4 uMAdult (>18 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)MaleNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)BothNormal details
SalivaDetected and Quantified0.944 +/- 0.442 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified1.24 +/- 0.728 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
UrineDetected and Quantified0.48-0.86 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified1.623 +/- 1.21 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified0.48-0.72 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified0.13 (0.02-0.45) umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified0.13 (0.03-0.34) umol/mmol creatinineAdult (>18 years old)FemaleNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1918 uMChildren (1-13 years old)Female
Maple syrup urine disease
    • Peritoneal dialys...
details
BloodDetected and Quantified0 uMAdult (>18 years old)FemaleBranched-chain Keto Acid Dehydrogenase Kinase Deficiency details
BloodDetected and Quantified0 uMAdolescent (13-18 years old)FemaleBranched-chain Keto Acid Dehydrogenase Kinase Deficiency details
Cerebrospinal Fluid (CSF)Detected and Quantified483 uMChildren (1-13 years old)Female
Maple syrup urine disease
    • Peritoneal dialys...
details
FecesDetected but not Quantified Adult (>18 years old)MaleGout details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
FecesDetected but not Quantified Adult (>18 years old)Femaleankylosing spondylitis details
FecesDetected but not Quantified Adult (>18 years old)Femalerheumatoid arthritis details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
UrineDetected and Quantified1.407 +/- 0.928 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
Associated Disorders and Diseases
Disease References
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 ]
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.
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]
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 ]
Eosinophilic esophagitis
  1. (). Mordechai, Hien, and David S. Wishart. .
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 ]
Associated OMIM IDs
DrugBank IDDB03229
Phenol Explorer Compound IDNot Available
FoodDB IDFDB012607
KNApSAcK IDC00019677
Chemspider ID69
KEGG Compound IDC00233
BioCyc ID2K-4CH3-PENTANOATE
BiGG ID34334
Wikipedia LinkNot Available
METLIN ID5663
PubChem Compound70
PDB IDCOI
ChEBI ID48430
References
Synthesis ReferenceTabuchi, Takeshi; Abe, Matazo. Fermentative preparation of a-ketoisocaproic acid. Jpn. Tokkyo Koho (1970), 3 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Martin PM, Gopal E, Ananth S, Zhuang L, Itagaki S, Prasad BM, Smith SB, Prasad PD, Ganapathy V: Identity of SMCT1 (SLC5A8) as a neuron-specific Na+-coupled transporter for active uptake of L-lactate and ketone bodies in the brain. J Neurochem. 2006 Jul;98(1):279-88. [PubMed:16805814 ]
  2. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
  3. Yudkoff M, Daikhin Y, Nissim I, Horyn O, Luhovyy B, Lazarow A, Nissim I: Brain amino acid requirements and toxicity: the example of leucine. J Nutr. 2005 Jun;135(6 Suppl):1531S-8S. [PubMed:15930465 ]
  4. Chow LS, Albright RC, Bigelow ML, Toffolo G, Cobelli C, Nair KS: Mechanism of insulin's anabolic effect on muscle: measurements of muscle protein synthesis and breakdown using aminoacyl-tRNA and other surrogate measures. Am J Physiol Endocrinol Metab. 2006 Oct;291(4):E729-36. Epub 2006 May 16. [PubMed:16705065 ]
  5. Wang Y, Holmes E, Nicholson JK, Cloarec O, Chollet J, Tanner M, Singer BH, Utzinger J: Metabonomic investigations in mice infected with Schistosoma mansoni: an approach for biomarker identification. Proc Natl Acad Sci U S A. 2004 Aug 24;101(34):12676-81. Epub 2004 Aug 16. [PubMed:15314235 ]
  6. Mitch WE, Walser M, Sapir DG: Nitrogen sparing induced by leucine compared with that induced by its keto analogue, alpha-ketoisocaproate, in fasting obese man. J Clin Invest. 1981 Feb;67(2):553-62. [PubMed:7462428 ]
  7. Sgaravatti AM, Rosa RB, Schuck PF, Ribeiro CA, Wannmacher CM, Wyse AT, Dutra-Filho CS, Wajner M: Inhibition of brain energy metabolism by the alpha-keto acids accumulating in maple syrup urine disease. Biochim Biophys Acta. 2003 Nov 20;1639(3):232-8. [PubMed:14636955 ]
  8. Schadewaldt P, Hammen HW, Ott AC, Wendel U: Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease. J Inherit Metab Dis. 1999 Aug;22(6):706-22. [PubMed:10472531 ]
  9. Hachey DL, Patterson BW, Reeds PJ, Elsas LJ: Isotopic determination of organic keto acid pentafluorobenzyl esters in biological fluids by negative chemical ionization gas chromatography/mass spectrometry. Anal Chem. 1991 May 1;63(9):919-23. [PubMed:1858984 ]

Enzymes

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
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
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
L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic aciddetails
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
L-Leucine + Oxoglutaric acid → Ketoleucine + L-Glutamic aciddetails