ContaminantDB: Ketoleucine

Identification Taxonomy Biological Properties Physical Properties Toxicity Profile Spectra Concentrations Links References XML

Record Information

Version

1.0

Creation Date

2014-08-29 06:51:13 UTC

Update Date

2026-04-02 23:25:41 UTC

Accession Number

CHEM003386

Identification

Common Name

Ketoleucine

Class

Small Molecule

Description

Ketoleucine is a metabolite that accumulates in Maple Syrup Urine Disease (MSUD) and shown to compromise brain energy metabolism by blocking the respiratory chain; this is of relevance to the understanding of the pathophysiology of the neurological dysfunction of MSUD patients. (1).

Contaminant Sources

EAFUS Chemicals
FooDB Chemicals
HMDB Contaminants - Feces
HMDB Contaminants - Urine
T3DB toxins

Contaminant Type

Animal Toxin
Food Toxin
Metabolite
Natural Compound
Organic Compound

Chemical 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 Mass

130.142 g/mol

Monoisotopic Mass

130.063 g/mol

CAS Registry Number

816-66-0

IUPAC Name

4-methyl-2-oxopentanoic acid

Traditional Name

ketoisocaproate

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 )

Biological Properties

Status

Detected and Not Quantified

Origin

Endogenous

Cellular Locations

Cytoplasm
Extracellular
Membrane
Mitochondria

Biofluid Locations

Not Available

Tissue Locations

Muscle
Neuron
Prostate

Pathways

Name	SMPDB Link	KEGG Link
Valine, Leucine and Isoleucine Degradation	SMP00032	map00280
Maple Syrup Urine Disease	SMP00199	Not Available

Applications

Not Available

Biological Roles

Chemical Roles

Not Available

Physical Properties

State

Liquid

Appearance

Not Available

Experimental Properties

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

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	1	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
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_2) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	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
LC-MS/MS	LC-MS/MS Spectrum - 35V, Negative	splash10-004i-0900000000-b66d521478c92e2d77d0	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 10V, Negative	splash10-004i-0900000000-9c3a698d9f28af574ef6	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 40V, Negative	splash10-03di-9000000000-47ced70de4860e900283	Spectrum
LC-MS/MS	LC-MS/MS Spectrum - 20V, Negative	splash10-03xr-7900000000-0461a5a56e36a03fe522	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
1D NMR	1H NMR Spectrum	Not Available	Spectrum
1D NMR	13C NMR Spectrum	Not Available	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
1D NMR	13C NMR Spectrum	Not Available	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
1D NMR	13C NMR Spectrum	Not Available	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
1D NMR	13C NMR Spectrum	Not Available	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
1D NMR	13C NMR Spectrum	Not Available	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
1D NMR	13C NMR Spectrum	Not Available	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
1D NMR	13C NMR Spectrum	Not Available	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
1D NMR	13C NMR Spectrum	Not Available	Spectrum
1D NMR	1H NMR Spectrum	Not Available	Spectrum
1D NMR	13C 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

Toxicity Profile

Route of Exposure

Not Available

Mechanism of Toxicity

Ketoleucine is a metabolite that accumulates in Maple Syrup Urine Disease (MSUD) and shown to compromise brain energy metabolism by blocking the respiratory chain.

Metabolism

Not Available

Toxicity Values

Not Available

Lethal Dose

Not Available

Carcinogenicity (IARC Classification)

No indication of carcinogenicity to humans (not listed by IARC).

Uses/Sources

This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation.

Minimum Risk Level

Not Available