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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (2) Show 2 proteins XML

Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2006-05-22 14:17:45 UTC

Update Date

2023-02-21 17:16:18 UTC

HMDB ID

HMDB0002266

Secondary Accession Numbers

HMDB0003520
HMDB02266
HMDB03520

Metabolite Identification

Common Name

(E)-2-Methylglutaconic acid

Description

2-Methylglutaconic acid is found in the urine of patients with organic aciduria from 2-methylacetoacetyl-CoA thiolase (EC 2.3.1.9, acetyl-CoA C-acetyltransferase) deficiency (ACAT, OMIM 607809 ). Main clinical features of ACAT include important staturo-ponderal delay, frequent infectious rhinopharyngitis episodes and an acute metabolic acidosis (this metabolic decompensation being adequately halted by bicarbonate supplementation). (PMID: 8930414 , 2925825 ).

Structure

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
(e)-2-Methylglutaconate	Generator
(e)-2-Methyl-2-pentenedioate	HMDB
(e)-2-Methyl-2-pentenedioic acid	HMDB
trans-2-Methylglutaconate	HMDB
trans-2-Methylglutaconic acid	HMDB
1-Methyl-1-propene-1,3-dicarboxylic acid	MeSH, HMDB
2-Methylglutaconic acid	MeSH, HMDB
2E-Methyl-glutaconate	Generator, HMDB
(e)-2-Methylglutaconic acid	MeSH

Chemical Formula

C₆H₈O₄

Average Molecular Weight

144.1253

Monoisotopic Molecular Weight

144.042258744

IUPAC Name

(2E)-2-methylpent-2-enedioic acid

Traditional Name

2E-methyl-glutaconic acid

CAS Registry Number

53358-21-7

SMILES

C\C(=C/CC(O)=O)C(O)=O

InChI Identifier

InChI=1S/C6H8O4/c1-4(6(9)10)2-3-5(7)8/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+

InChI Key

JKGHDBJDBRBRNA-DUXPYHPUSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as methyl-branched fatty acids. These are fatty acids with an acyl chain that has a methyl branch. Usually, they are saturated and contain only one or more methyl group. However, branches other than methyl may be present.

Kingdom

Organic compounds

Super Class

Lipids and lipid-like molecules

Class

Fatty Acyls

Sub Class

Fatty acids and conjugates

Direct Parent

Methyl-branched fatty acids

Alternative Parents

Substituents

Methyl-branched fatty acid
Unsaturated fatty acid
Dicarboxylic acid or derivatives
Carboxylic acid
Carboxylic acid derivative
Organic oxygen compound
Organic oxide
Hydrocarbon derivative
Organooxygen compound
Carbonyl group
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

Dicarboxylic acids (LMFA01170079 )

Ontology

Physiological effect

Not Available

Disposition

Biological location

Cellular substructure

Organelle

Adiposome (HMDB: HMDB0002266)

Excreta

Biofluid or Excreta

Urine (HMDB: HMDB0002266)

Route of exposure

Enteral

Ingestion (HMDB: HMDB0002266)

Source

Endogenous

Endogenous (HMDB: HMDB0002266)

Animal

Animal (HMDB: HMDB0002266)

Exogenous

Food

Food (HMDB: HMDB0002266)

Animal origin

Poultry

Greylag goose (FooDB: FOOD00368)
Guinea hen (FooDB: FOOD00373)
Ostrich (FooDB: FOOD00422)
Velvet duck (FooDB: FOOD00427)
Pheasant (FooDB: FOOD00430)
Squab (FooDB: FOOD00481)
Turkey (FooDB: FOOD00494)
Quail (FooDB: FOOD00541)
Anatidae (FooDB: FOOD00576)
Rock ptarmigan (FooDB: FOOD00597)
Columbidae (Dove, Pigeon) (FooDB: FOOD00610)
Chicken (FooDB: FOOD00329)
Mallard duck (FooDB: FOOD00353)
Emu (FooDB: FOOD00360)

Equine

Horse (FooDB: FOOD00378)

Lagomorph

Ovis

Sheep (Mutton, Lamb) (FooDB: FOOD00473)

Bovine

Venison

Deer (FooDB: FOOD00524)
Mule deer (FooDB: FOOD00348)
Elk (FooDB: FOOD00359)

Caprae

Domestic goat (FooDB: FOOD00529)

Swine

Domestic pig (FooDB: FOOD00536)
Wild boar (FooDB: FOOD00307)

Process

Naturally occurring process

Biological process

Biochemical pathway

Metabolic pathway

Fatty Acid Metabolism (HMDB: HMDB0002266)

Chemical reaction

Lipid Peroxidation (HMDB: HMDB0002266)

Cellular process

Cell signaling (HMDB: HMDB0002266)

Biochemical process

Lipid transport (HMDB: HMDB0002266)

Role

Biological role

Energy storage (HMDB: HMDB0002266)

Industrial application

Manufacturing industry

Material processing agent

Fluid processing agent

Surfactant

Surfactant (HMDB: HMDB0002266)

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	Not Available	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	Not Available	Not Available
LogP	Not Available	Not Available

Experimental Chromatographic Properties

Not Available

Predicted Molecular Properties

Property	Value	Source
Water Solubility	8.75 g/L	ALOGPS
logP	0.3	ALOGPS
logP	0.44	ChemAxon
logS	-1.2	ALOGPS
pKa (Strongest Acidic)	3.84	ChemAxon
Physiological Charge	-2	ChemAxon
Hydrogen Acceptor Count	4	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	74.6 Å²	ChemAxon
Rotatable Bond Count	3	ChemAxon
Refractivity	33.59 m³·mol⁻¹	ChemAxon
Polarizability	13.13 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

Predicted Chromatographic Properties

Predicted Collision Cross Sections

Predictor	Adduct Type	CCS Value (Å²)	Reference
DarkChem	[M+H]+	133.119	31661259
DarkChem	[M-H]-	126.237	31661259
DeepCCS	[M+H]+	130.831	30932474
DeepCCS	[M-H]-	127.01	30932474
DeepCCS	[M-2H]-	164.311	30932474
DeepCCS	[M+Na]+	139.506	30932474
AllCCS	[M+H]+	133.0	32859911
AllCCS	[M+H-H2O]+	128.9	32859911
AllCCS	[M+NH4]+	136.8	32859911
AllCCS	[M+Na]+	137.9	32859911
AllCCS	[M-H]-	127.9	32859911
AllCCS	[M+Na-2H]-	130.1	32859911
AllCCS	[M+HCOO]-	132.6	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
(E)-2-Methylglutaconic acid	C\C(=C/CC(O)=O)C(O)=O	2434.1	Standard polar	33892256
(E)-2-Methylglutaconic acid	C\C(=C/CC(O)=O)C(O)=O	1119.0	Standard non polar	33892256
(E)-2-Methylglutaconic acid	C\C(=C/CC(O)=O)C(O)=O	1385.6	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
(E)-2-Methylglutaconic acid,1TMS,isomer #1	C/C(=C\CC(=O)O[Si](C)(C)C)C(=O)O	1424.1	Semi standard non polar	33892256
(E)-2-Methylglutaconic acid,1TMS,isomer #2	C/C(=C\CC(=O)O)C(=O)O[Si](C)(C)C	1398.8	Semi standard non polar	33892256
(E)-2-Methylglutaconic acid,2TMS,isomer #1	C/C(=C\CC(=O)O[Si](C)(C)C)C(=O)O[Si](C)(C)C	1497.0	Semi standard non polar	33892256
(E)-2-Methylglutaconic acid,1TBDMS,isomer #1	C/C(=C\CC(=O)O[Si](C)(C)C(C)(C)C)C(=O)O	1677.2	Semi standard non polar	33892256
(E)-2-Methylglutaconic acid,1TBDMS,isomer #2	C/C(=C\CC(=O)O)C(=O)O[Si](C)(C)C(C)(C)C	1656.9	Semi standard non polar	33892256
(E)-2-Methylglutaconic acid,2TBDMS,isomer #1	C/C(=C\CC(=O)O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	1925.9	Semi standard non polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted GC-MS	Predicted GC-MS Spectrum - (E)-2-Methylglutaconic acid GC-MS (Non-derivatized) - 70eV, Positive	splash10-0f6w-9200000000-3c26a0f7a91d6acef0f0	2017-09-01	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (E)-2-Methylglutaconic acid GC-MS (2 TMS) - 70eV, Positive	splash10-00di-9320000000-06523487fcb76b7974f7	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (E)-2-Methylglutaconic acid GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (E)-2-Methylglutaconic acid 10V, Positive-QTOF	splash10-002b-4900000000-a4f5b1870a7ab5d7d9d7	2016-08-03	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (E)-2-Methylglutaconic acid 20V, Positive-QTOF	splash10-0032-9300000000-8690078e13fde4da86e9	2016-08-03	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (E)-2-Methylglutaconic acid 40V, Positive-QTOF	splash10-0udi-9000000000-3c72687cfe5825fe8620	2016-08-03	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (E)-2-Methylglutaconic acid 10V, Negative-QTOF	splash10-0006-3900000000-eeb6bed3f0b5c36c6f79	2016-08-03	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (E)-2-Methylglutaconic acid 20V, Negative-QTOF	splash10-0005-9400000000-650d0aa40bf2dd38635f	2016-08-03	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (E)-2-Methylglutaconic acid 40V, Negative-QTOF	splash10-0a59-9100000000-a82ead692dae80600526	2016-08-03	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (E)-2-Methylglutaconic acid 10V, Positive-QTOF	splash10-001i-9200000000-624b23b748d39e75d3c4	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (E)-2-Methylglutaconic acid 20V, Positive-QTOF	splash10-001i-9000000000-ebe23e1d52795153d360	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (E)-2-Methylglutaconic acid 40V, Positive-QTOF	splash10-0udi-9000000000-1af77fc95f979fc3b380	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (E)-2-Methylglutaconic acid 10V, Negative-QTOF	splash10-002b-9600000000-be26d3e872704242dd9b	2021-09-23	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (E)-2-Methylglutaconic acid 20V, Negative-QTOF	splash10-00ea-9000000000-035726473be11e09463d	2021-09-23	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (E)-2-Methylglutaconic acid 40V, Negative-QTOF	splash10-001i-9000000000-b8174f664b8393ce68d6	2021-09-23	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum

IR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted IR Spectrum	IR Ion Spectrum (Quadrupole Ion Trap, ESI+, Adduct: [M+H]+)	2022-02-11	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)	2023-02-03	FELIX lab	View Spectrum

Biological Properties

Cellular Locations

Extracellular
Membrane

Biospecimen Locations

Urine

Tissue Locations

Not Available

Pathways

Not Available

Name	SMPDB/PathBank	KEGG

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Urine	Detected and Quantified	<2 umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	BC Children's Hos...	details

Abnormal Concentrations

Not Available

Associated Disorders and Diseases

Disease References

None

Associated OMIM IDs

None

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022936

KNApSAcK ID

Not Available

Chemspider ID

4897953

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

6584

PubChem Compound

6368126

PDB ID

Not Available

ChEBI ID

Not Available

Food Biomarker Ontology

Not Available

VMH ID

CE5068

MarkerDB ID

Not Available

Good Scents ID

Not Available

References

Synthesis Reference

Kagan, Jacques; Tolentino, Luisito; Ettlinger, Martin G. Solution of a classical problem. Tautomerism and isomerism in the a-methylglutaconic acid series. Journal of Organic Chemistry (1975), 40(21), 3085-93.

Material Safety Data Sheet (MSDS)

Not Available

General References

Duran M, Bruinvis L, Ketting D, Kamerling JP, Wadman SK, Schutgens RB: The identification of (E)-2-methylglutaconic acid, a new isoleucine metabolite, in the urine of patients with beta-ketothiolase deficiency, propionic acidaemia and methylmalonic acidaemia. Biomed Mass Spectrom. 1982 Jan;9(1):1-5. [PubMed:7059658 ]
Lefevere MF, Verhaeghe BJ, Declerck DH, Van Bocxlaer JF, De Leenheer AP, De Sagher RM: Metabolic profiling of urinary organic acids by single and multicolumn capillary gas chromatography. J Chromatogr Sci. 1989 Jan;27(1):23-9. [PubMed:2925825 ]
Fontaine M, Briand G, Ser N, Armelin I, Rolland MO, Degand P, Vamecq J: Metabolic studies in twin brothers with 2-methylacetoacetyl-CoA thiolase deficiency. Clin Chim Acta. 1996 Nov 15;255(1):67-83. [PubMed:8930414 ]

Enzymes

Enzyme Details

1. Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

General function:: Involved in ligase activity
Specific function:: Not Available
Gene Name:: MCCC2
Uniprot ID:: Q9HCC0
Molecular weight:: 61332.65

References

Duran M, Bruinvis L, Ketting D, Kamerling JP, Wadman SK, Schutgens RB: The identification of (E)-2-methylglutaconic acid, a new isoleucine metabolite, in the urine of patients with beta-ketothiolase deficiency, propionic acidaemia and methylmalonic acidaemia. Biomed Mass Spectrom. 1982 Jan;9(1):1-5. [PubMed:7059658 ]

Enzyme Details

2. Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

General function:: Involved in catalytic activity
Specific function:: Not Available
Gene Name:: MCCC1
Uniprot ID:: Q96RQ3
Molecular weight:: 80472.45

References

Duran M, Bruinvis L, Ketting D, Kamerling JP, Wadman SK, Schutgens RB: The identification of (E)-2-methylglutaconic acid, a new isoleucine metabolite, in the urine of patients with beta-ketothiolase deficiency, propionic acidaemia and methylmalonic acidaemia. Biomed Mass Spectrom. 1982 Jan;9(1):1-5. [PubMed:7059658 ]

Showing metabocard for (E)-2-Methylglutaconic acid (HMDB0002266)

MOL for HMDB0002266 ((E)-2-Methylglutaconic acid)

3D MOL for HMDB0002266 ((E)-2-Methylglutaconic acid)

3D SDF for HMDB0002266 ((E)-2-Methylglutaconic acid)

3D-SDF for HMDB0002266 ((E)-2-Methylglutaconic acid)

PDB for HMDB0002266 ((E)-2-Methylglutaconic acid)

3D PDB for HMDB0002266 ((E)-2-Methylglutaconic acid)

SMILES for HMDB0002266 ((E)-2-Methylglutaconic acid)

INCHI for HMDB0002266 ((E)-2-Methylglutaconic acid)

Structure for HMDB0002266 ((E)-2-Methylglutaconic acid)

3D Structure for HMDB0002266 ((E)-2-Methylglutaconic acid)

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra

IR Spectra

Enzymes

References

References