Human Metabolome Database: Showing metabocard for Ketoleucine (HMDB0000695)

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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (4) Show 4 proteins XML

enzymes (4) Show 4 proteins

Record Information

Version

4.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2020-02-27 20:30:46 UTC

HMDB ID

HMDB0000695

Secondary Accession Numbers

HMDB0000762
HMDB00695
HMDB00762

Metabolite Identification

Common Name

Ketoleucine

Description

Ketoleucine 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

MOL SDF PDB SMILES InChI

Synonyms

Value	Source
2-oxo-4-METHYLPENTANOIC ACID	ChEBI
2-Oxoisocaproate	ChEBI
4-Methyl-2-oxopentanoate	ChEBI
4-Methyl-2-oxovaleric acid	ChEBI
alpha-Ketoisocaproic acid	ChEBI
2-oxo-4-METHYLPENTANOate	Generator
2-Oxoisocaproic acid	Generator
4-Methyl-2-oxopentanoic acid	Generator
4-Methyl-2-oxovalerate	Generator
a-Ketoisocaproate	Generator
a-Ketoisocaproic acid	Generator
alpha-Ketoisocaproate	Generator
Α-ketoisocaproate	Generator
Α-ketoisocaproic acid	Generator
2-Keto-4-methylvalerate	HMDB
2-Keto-4-methylvaleric acid	HMDB
2-Ketoisocaproate	HMDB
2-Ketoisocaproic acid	HMDB
2-oxo-4-Methylvalerate	HMDB
2-oxo-4-Methylvaleric acid	HMDB
2-Oxoleucine	HMDB
4-Methyl-2-oxo-valerate	HMDB
4-Methyl-2-oxo-valeric acid	HMDB
a-Ketoisocapronate	HMDB
a-Ketoisocapronic acid	HMDB
a-Oxoisocaproate	HMDB
a-Oxoisocaproic acid	HMDB
alpha-Keto-isocaproate	HMDB
alpha-Keto-isocaproic acid	HMDB
alpha-Ketoisocapronate	HMDB
alpha-Ketoisocapronic acid	HMDB
alpha-Oxoisocaproate	HMDB
alpha-Oxoisocaproic acid	HMDB
Ketoisocaproate	HMDB
Ketoisocaproic acid	HMDB
Methyloxovalerate	HMDB
Methyloxovaleric acid	HMDB
Oxoisocaproate	HMDB
Oxoisocaproic acid	HMDB
Keto-leucine	HMDB

Chemical Formula

C₆H₁₀O₃

Average Molecular Weight

130.1418

Monoisotopic Molecular Weight

130.062994186

IUPAC Name

4-methyl-2-oxopentanoic acid

Traditional Name

ketoisocaproate

CAS Registry Number

816-66-0

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 Key

BKAJNAXTPSGJCU-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.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Keto acids and derivatives

Sub Class

Short-chain keto acids and derivatives

Direct Parent

Short-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 Framework

Aliphatic acyclic compounds

External Descriptors

2-oxo monocarboxylic acid (CHEBI:48430 )

Ontology

Ingestion

Source:

Biological:

Animal

Plant:

Biological location:

Process

Naturally occurring process:

Biological process:

Cellular process:

Cell signaling

Biochemical pathway:

Valine, Leucine and Isoleucine Degradation

Role

Indirect biological role:

Biological role:

Industrial application:

Pharmaceutical industry:

Pharmaceutical

Physical Properties

State

Liquid

Experimental Properties

Property	Value	Reference
Melting Point	8 - 10 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	32 mg/mL	Not Available
LogP	Not Available	Not Available

Predicted Properties

Property	Value	Source
Water Solubility	6.76 g/L	ALOGPS
logP	0.82	ALOGPS
logP	1.5	ChemAxon
logS	-1.3	ALOGPS
pKa (Strongest Acidic)	3.53	ChemAxon
pKa (Strongest Basic)	-9.7	ChemAxon
Physiological Charge	-1	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	1	ChemAxon
Polar Surface Area	54.37 Å²	ChemAxon
Rotatable Bond Count	3	ChemAxon
Refractivity	31.77 m³·mol⁻¹	ChemAxon
Polarizability	13 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Spectra

Spectrum Type	Description	Splash Key	View
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)	splash10-000i-9400000000-49d055f04bfe1877ac5b	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)	splash10-029j-8930000000-c6c54bf6ee2995a2195b	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-0002-1910000000-0ba38257e25873312809	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (2 TMS)	splash10-0a5c-4930000000-61247369a805fa2a77a7	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (1 MEOX; 1 TMS)	splash10-000i-9510000000-8bfc5a54e29f65edf361	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (1 MEOX; 1 TMS)	splash10-00lj-9620000000-aa8409ceb2359d903f38	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-000i-9400000000-49d055f04bfe1877ac5b	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-029j-8930000000-c6c54bf6ee2995a2195b	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0002-1910000000-0ba38257e25873312809	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-0a5c-4930000000-61247369a805fa2a77a7	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-000i-9510000000-8bfc5a54e29f65edf361	Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-00lj-9620000000-aa8409ceb2359d903f38	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-00ks-7930000000-4e9cfd715fa884d30e7f	Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-000i-9500000000-709ebcb57f006703c269	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-0006-9000000000-e410bce23706f826f8dd	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positive	splash10-0006-9400000000-c4f06cea1e27a2b24660	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)	splash10-0006-9000000000-bed7ac074a60210971e3	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)	splash10-014i-9200000000-2fa08d2b4df406cf63ea	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)	splash10-014j-9000000000-045be8e8dca3f93d45ba	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative	splash10-004i-0900000000-237fa8ad6bfb929be31e	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative	splash10-004r-9700000000-8d38d24fce78b1ff13e6	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative	splash10-0a59-9000000000-759cfc0817917283d1a5	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative	splash10-0a4l-9000000000-2a068ada71fbe0a5a988	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative	splash10-0a4l-9000000000-58e61e31f3c96d84d799	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-004i-0900000000-237fa8ad6bfb929be31e	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-004r-9700000000-8d38d24fce78b1ff13e6	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-0a59-9000000000-759cfc0817917283d1a5	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-0a4l-9000000000-2a068ada71fbe0a5a988	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-0a4l-9000000000-58e61e31f3c96d84d799	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT , negative	splash10-004i-0900000000-556a0d1cf568609752d1	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-IT , negative	splash10-004r-5900000000-20f6923c8ed45e9fa088	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-08gr-7900000000-31021fda89eb6535185f	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-0btm-9200000000-3bf5df4b557ef87267f4	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-0a4i-9000000000-37839bf70647d24b94a4	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-004i-4900000000-796467909d1d4c087a07	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-01ri-9400000000-0abcd021721f32c10e42	Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-015l-9000000000-5c33a47dfeb09a182983	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
1D NMR	13C NMR Spectrum	Not Available	Spectrum
2D NMR	[1H,13C] 2D NMR Spectrum	Not Available	Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Extracellular
Membrane
Mitochondria

Biospecimen Locations

Blood
Cerebrospinal Fluid (CSF)
Feces
Saliva
Urine

Tissue Locations

Muscle
Neuron
Placenta
Prostate

Pathways

Name	SMPDB/Pathwhiz	KEGG
Valine, Leucine and Isoleucine Degradation
Beta-Ketothiolase Deficiency		Not Available
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency		Not Available
Propionic Acidemia		Not Available
3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	28.0 (0.0-58.0) uM	Adult (>18 years old)	Both	Normal	8412012	details
Blood	Detected but not Quantified		Adult (>18 years old)	Female	Normal	27355821	details
Blood	Detected and Quantified	33.5 +/- 8.2 uM	Adult (>18 years old)	Both	Normal	Geigy Scientific ...	details
Blood	Detected and Quantified	25-28 uM	Adolescent (13-18 years old)	Female	Normal	22956686	details
Blood	Detected and Quantified	18 uM	Adult (>18 years old)	Female	Normal	22956686	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	5.0 +/- 4.4 uM	Adult (>18 years old)	Both	Normal	8412012	details
Feces	Detected but not Quantified		Adult (>18 years old)	Male	Normal	28270806	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	25037050	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	27543620	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	29808030	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	18052033	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	18085514	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Normal	25670064	details
Saliva	Detected and Quantified	0.944 +/- 0.442 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	1.24 +/- 0.728 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Normal	22308371	details
Urine	Detected and Quantified	0-2 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	31506554	details
Urine	Detected and Quantified	0.48-0.86 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected and Quantified	1.623 +/- 1.21 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Mordechai, Hien, ...	details
Urine	Detected and Quantified	0.48-0.72 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected and Quantified	0-0.1 umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	31963255	details
Urine	Detected and Quantified	0-0.2 umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	31963255	details
Urine	Detected and Quantified	0-0.7 umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	31963255	details
Urine	Detected and Quantified	0.13 (0.02-0.45) umol/mmol creatinine	Adult (>18 years old)	Male	Normal	9869358	details
Urine	Detected and Quantified	0.13 (0.03-0.34) umol/mmol creatinine	Adult (>18 years old)	Female	Normal	9869358	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	1918 uM	Children (1-13 years old)	Female	Maple syrup urine disease	Peritoneal dialys...	details
Blood	Detected and Quantified	0 uM	Adult (>18 years old)	Female	Branched-chain Keto Acid Dehydrogenase Kinase Deficiency	22956686	details
Blood	Detected and Quantified	0 uM	Adolescent (13-18 years old)	Female	Branched-chain Keto Acid Dehydrogenase Kinase Deficiency	22956686	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	483 uM	Children (1-13 years old)	Female	Maple syrup urine disease	Peritoneal dialys...	details
Feces	Detected but not Quantified		Adult (>18 years old)	Male	Gout	28270806	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Colorectal cancer	27015276	details
Feces	Detected but not Quantified		Adult (>18 years old)	Female	ankylosing spondylitis	Tie-juan Sha...	details
Feces	Detected but not Quantified		Adult (>18 years old)	Female	rheumatoid arthritis	Tie-juan Sha...	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Colorectal Cancer	27275383	details
Feces	Detected but not Quantified		Adult (>18 years old)	Both	Colorectal cancer	25037050	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Attachment loss	31026179	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Missing teeth	31026179	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Periodontal Probing Depth	31026179	details
Saliva	Detected but not Quantified		Adult (>18 years old)	Male	Tooth Decay	31026179	details
Urine	Detected and Quantified	1.407 +/- 0.928 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Mordechai, Hien, ...	details

Associated Disorders and Diseases

Disease References

Maple syrup urine disease
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
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
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 ] 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 ] 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
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
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
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
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
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
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
(). Mordechai, Hien, and David S. Wishart. .

Associated OMIM IDs

248600 (Maple syrup urine disease)
614923 (Branched-chain Keto Acid Dehydrogenase Kinase Deficiency)
114500 (Colorectal cancer)
138900 (Gout)
180300 (Rheumatoid arthritis)
610247 (Eosinophilic esophagitis)

External Links

DrugBank ID

DB03229

Phenol Explorer Compound ID

Not Available

FoodDB ID

FDB012607

KNApSAcK ID

C00019677

Chemspider ID

KEGG Compound ID

C00233

BioCyc ID

2K-4CH3-PENTANOATE

BiGG ID

34334

Wikipedia Link

Not Available

METLIN ID

5663

PubChem Compound

PDB ID

Not Available

ChEBI ID

48430

Food Biomarker Ontology

Not Available

VMH ID

Not Available

References

Synthesis Reference

Tabuchi, Takeshi; Abe, Matazo. Fermentative preparation of a-ketoisocaproic acid. Jpn. Tokkyo Koho (1970), 3 pp.

Material Safety Data Sheet (MSDS)

Download (PDF)

General References

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 ]
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 ]
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 ]
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 ]
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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 dioxide	details

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 dioxide	details

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

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

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

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

Showing metabocard for Ketoleucine (HMDB0000695)

Structure for HMDB0000695 (Ketoleucine)

Enzymes

Reactions

Reactions

Reactions

Reactions