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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

2005-11-16 15:48:42 UTC

Update Date

2020-11-09 23:11:56 UTC

HMDB ID

HMDB0000072

Secondary Accession Numbers

HMDB0000461
HMDB00072
HMDB00461

Metabolite Identification

Common Name

cis-Aconitic acid

Description

cis-Aconitic acid, also known as cis-aconitate or acid, acontic, belongs to the class of organic compounds known as tricarboxylic acids and derivatives. These are carboxylic acids containing exactly three carboxyl groups. cis-Aconitic acid is a moderately acidic compound (based on its pKa). cis-Aconitic acid exists in all living species, ranging from bacteria to humans. Within humans, cis-aconitic acid participates in a number of enzymatic reactions. In particular, cis-aconitic acid can be biosynthesized from citric acid through the action of the enzyme aconitate hydratase, mitochondrial. In addition, cis-aconitic acid can be converted into isocitric acid; which is mediated by the enzyme aconitate hydratase, mitochondrial. In humans, cis-aconitic acid is involved in the metabolic disorder called the glutaminolysis and cancer pathway. cis-Aconitic acid is a bland tasting compound. Outside of the human body, cis-aconitic acid has been detected, but not quantified in, several different foods, such as jackfruits, root vegetables, breakfast cereals, oval-leaf huckleberries, and bog bilberries. This could make cis-aconitic acid a potential biomarker for the consumption of these foods.

Structure

MOL SDF 3D SDF PDB 3D PDB SMILES InChI

HEADER    PROTEIN                                 27-MAY-19   NONE
TITLE     NULL                                                        
COMPND    MOLECULE: cis-Aconitic acid.mol                             
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   27-MAY-19         0                                  
HETATM    1  C   UNK     0       2.667  -0.770   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0       1.334  -1.540   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0       0.000  -0.770   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0       0.000   0.770   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0      -1.334  -1.540   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0      -2.667  -0.770   0.000  0.00  0.00           C+0
HETATM    7  O   UNK     0       2.667   0.770   0.000  0.00  0.00           O+0
HETATM    8  O   UNK     0       4.001  -1.540   0.000  0.00  0.00           O+0
HETATM    9  O   UNK     0      -1.334   1.540   0.000  0.00  0.00           O+0
HETATM   10  O   UNK     0       1.334   1.540   0.000  0.00  0.00           O+0
HETATM   11  O   UNK     0      -2.667   0.770   0.000  0.00  0.00           O+0
HETATM   12  O   UNK     0      -4.001  -1.540   0.000  0.00  0.00           O+0
CONECT    1    2    7    8                                                  
CONECT    2    1    3                                                       
CONECT    3    2    4    5                                                  
CONECT    4    3    9   10                                                  
CONECT    5    3    6                                                       
CONECT    6    5   11   12                                                  
CONECT    7    1                                                            
CONECT    8    1                                                            
CONECT    9    4                                                            
CONECT   10    4                                                            
CONECT   11    6                                                            
CONECT   12    6                                                            
MASTER        0    0    0    0    0    0    0    0   12    0   22    0
END

COMPND    https://cactus.nci.nih.gov/chemical/structure/OC(=O)C%5CC(=C%5CC(O)=O)C(O)=O/... 
AUTHOR    GENERATED BY OPEN BABEL 3.1.0
HETATM    1  O   UNL     1       3.745  -0.669   0.188  1.00  0.00           O  
HETATM    2  C   UNL     1       2.445  -0.456  -0.066  1.00  0.00           C  
HETATM    3  O   UNL     1       2.047  -0.437  -1.207  1.00  0.00           O  
HETATM    4  C   UNL     1       1.480  -0.240   1.071  1.00  0.00           C  
HETATM    5  C   UNL     1       0.094  -0.025   0.521  1.00  0.00           C  
HETATM    6  C   UNL     1      -0.756  -1.059   0.419  1.00  0.00           C  
HETATM    7  C   UNL     1      -2.157  -0.826   0.015  1.00  0.00           C  
HETATM    8  O   UNL     1      -2.973  -1.872  -0.220  1.00  0.00           O  
HETATM    9  O   UNL     1      -2.573   0.308  -0.105  1.00  0.00           O  
HETATM   10  C   UNL     1      -0.329   1.323   0.094  1.00  0.00           C  
HETATM   11  O   UNL     1      -0.283   1.662  -1.210  1.00  0.00           O  
HETATM   12  O   UNL     1      -0.718   2.127   0.917  1.00  0.00           O  
HETATM   13  H   UNL     1       4.325  -0.803  -0.574  1.00  0.00           H  
HETATM   14  H   UNL     1       1.481  -1.117   1.720  1.00  0.00           H  
HETATM   15  H   UNL     1       1.784   0.636   1.644  1.00  0.00           H  
HETATM   16  H   UNL     1      -0.416  -2.062   0.632  1.00  0.00           H  
HETATM   17  H   UNL     1      -3.881  -1.670  -0.482  1.00  0.00           H  
HETATM   18  H   UNL     1      -0.570   2.556  -1.441  1.00  0.00           H  
CONECT    1    2   13                                                 
CONECT    2    1    3    3    4                                       
CONECT    3    2    2                                                 
CONECT    4    2    5   14   15                                       
CONECT    5    4    6    6   10                                       
CONECT    6    5    5    7   16                                       
CONECT    7    6    8    9    9                                       
CONECT    8    7   17                                                 
CONECT    9    7    7                                                 
CONECT   10    5   11   12   12                                       
CONECT   11   10   18                                                 
CONECT   12   10   10                                                 
CONECT   13    1                                                      
CONECT   14    4                                                      
CONECT   15    4                                                      
CONECT   16    6                                                      
CONECT   17    8                                                      
CONECT   18   11                                                      
MASTER        0    0    0    0    0    0    0    0   18    0   18    0
END

View in JSmol View Stereo Labels

Synonyms

Value	Source
(Z)-1-Propene-1,2,3-tricarboxylic acid	ChEBI
cis-1-Propene-1,2,3-tricarboxylic acid	ChEBI
(Z)-1-Propene-1,2,3-tricarboxylate	Generator
cis-1-Propene-1,2,3-tricarboxylate	Generator
cis-Aconitate	Generator
(1Z)-1-Propene-1,2,3-tricarboxylate	HMDB
(1Z)-1-Propene-1,2,3-tricarboxylic acid	HMDB
(Z)-Aconitate	HMDB
(Z)-Aconitic acid	HMDB
1-cis-2,3-Propenetricarboxylate	HMDB
1-cis-2,3-Propenetricarboxylic acid	HMDB
1-Propene-1,2,3-tricarboxylate	HMDB
1-Propene-1,2,3-tricarboxylic acid	HMDB
cis-Aconate	HMDB
cis-Aconic acid	HMDB
cis-Oxaloacetate	HMDB
cis-Oxaloacetic acid	HMDB
Acid, citridic	HMDB
Acid, citridinic	HMDB
Aconitic acid	HMDB
Acontic acid	HMDB
Adonic acid	HMDB
Achilleic acid	HMDB
Acid, acontic	HMDB
Acid, equisetic	HMDB
Citridinic acid	HMDB
Acid, achilleic	HMDB
Aconitate	HMDB
Citridic acid	HMDB
Pyrocitric acid	HMDB
Acid, aconitic	HMDB
Acid, adonic	HMDB
Acid, carboxyglutaconic	HMDB
Acid, pyrocitric	HMDB
Carboxyglutaconic acid	HMDB
Equisetic acid	HMDB

Chemical Formula

C₆H₆O₆

Average Molecular Weight

174.1082

Monoisotopic Molecular Weight

174.016437924

IUPAC Name

(1Z)-prop-1-ene-1,2,3-tricarboxylic acid

Traditional Name

cis-aconitic acid

CAS Registry Number

585-84-2

SMILES

OC(=O)C\C(=C\C(O)=O)C(O)=O

InChI Identifier

InChI=1S/C6H6O6/c7-4(8)1-3(6(11)12)2-5(9)10/h1H,2H2,(H,7,8)(H,9,10)(H,11,12)/b3-1-

InChI Key

GTZCVFVGUGFEME-IWQZZHSRSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as tricarboxylic acids and derivatives. These are carboxylic acids containing exactly three carboxyl groups.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Tricarboxylic acids and derivatives

Direct Parent

Tricarboxylic acids and derivatives

Alternative Parents

Substituents

Tricarboxylic acid or derivatives
Carboxylic acid
Organic oxygen compound
Organic oxide
Hydrocarbon derivative
Organooxygen compound
Carbonyl group
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

aconitic acid (CHEBI:32805 )

Ontology

Physiological effect

Health effect:

Health condition:

Schizophrenia

Cancer:

Lung cancer

Disposition

Route of exposure:

Enteral:

Ingestion

Source:

Biological:

Plant:

Animal

Biological location:

Organ and components:

Prostate

Biofluid and excreta:

Subcellular:

Process

Naturally occurring process:

Biological process:

Biochemical pathway:

Physical Properties

State

Solid

Experimental Properties

Property	Value	Reference
Melting Point	125 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	400 mg/mL	Not Available
LogP	Not Available	Not Available

Predicted Properties

Property	Value	Source
Water Solubility	6.72 g/L	ALOGPS
logP	-0.41	ALOGPS
logP	-0.52	ChemAxon
logS	-1.4	ALOGPS
pKa (Strongest Acidic)	2.11	ChemAxon
Physiological Charge	-3	ChemAxon
Hydrogen Acceptor Count	6	ChemAxon
Hydrogen Donor Count	3	ChemAxon
Polar Surface Area	111.9 Å²	ChemAxon
Rotatable Bond Count	4	ChemAxon
Refractivity	35.23 m³·mol⁻¹	ChemAxon
Polarizability	14.16 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

GC-MS

Spectrum Type	Description	Splash Key	Deposition Date	View
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)	splash10-0002-1940000000-24c70788b42e3006de46	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-0002-0940000000-3ab0a8161f2ec8f04452	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (3 TMS)	splash10-00dj-9630000000-1dc322333cb48ef39588	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (3 TMS)	splash10-004i-3591000000-a58ec1ba633b098e0867	2014-06-16	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0002-1940000000-24c70788b42e3006de46	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-0002-0940000000-3ab0a8161f2ec8f04452	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-EI-TOF (Non-derivatized)	splash10-00dj-9630000000-1dc322333cb48ef39588	2017-09-12	View Spectrum
GC-MS	GC-MS Spectrum - GC-MS (Non-derivatized)	splash10-004i-3591000000-a58ec1ba633b098e0867	2017-09-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-06w9-4900000000-38239492ad0e66d6533b	2016-09-22	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (3 TMS) - 70eV, Positive	splash10-00di-9167000000-07ccdac5775cc3e75c95	2017-10-06	View Spectrum
MS	Mass Spectrum (Electron Ionization)	splash10-004i-3900000000-b23042db4fd2074815f9	2021-09-05	View Spectrum

LC-MS/MS

Spectrum Type	Description	Splash Key	Deposition Date	View
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)	splash10-000i-9300000000-1f04239a9a7133f89d08	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)	splash10-000f-9000000000-49f5c2a7959cfbbbe09f	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)	splash10-000f-9100000000-d4857c30b96cf4786dbe	2012-07-24	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-00di-0911000000-3197b86ab2053b968bf9	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-001i-9000000000-74977ead259f739d154b	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-004i-0900000000-9f4cc240b2470f2bbb93	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-001i-0900000000-5034c256b831c6173452	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-00di-0912000000-a603cd799b70c1089ddd	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-001i-9000000000-ce8dbec6a7b0d83b372c	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-004i-0900000000-927f79de2cf5ffcb2c52	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative	splash10-003r-0194000000-75d834c69fbfd0a2f7be	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative	splash10-00fr-0900000000-aad30bb884e0e9a5f01c	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative	splash10-000i-9200000000-e90e540bcd0659005472	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative	splash10-000i-9000000000-0edcc061c37b1fbb9fa1	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative	splash10-000l-9000000000-1e762d38e4e5cabfcb6b	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative	splash10-000f-9000000000-5602736cc28db9eb9085	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative	splash10-000i-9400000000-c8ec5b3e604b870eab95	2012-08-31	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-00fr-0900000000-aad30bb884e0e9a5f01c	2017-09-14	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QQ , negative	splash10-000i-9200000000-e90e540bcd0659005472	2017-09-14	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-004i-0900000000-8e26562af9ae45eb3303	2016-09-12	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-02di-1900000000-7fec32a5e34c3b55d683	2016-09-12	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-01w0-9600000000-ee172c3013370ab0da31	2016-09-12	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Negative	splash10-00fr-1900000000-18635ce5a20ce1b78139	2016-09-12	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Negative	splash10-00b9-2900000000-58bb75a2a1b28dbffd73	2016-09-12	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Negative	splash10-06rf-9300000000-19df730d01f07fcf6573	2016-09-12	View Spectrum

NMR

Spectrum Type	Description	Deposition Date	View
1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, experimental)	2012-12-04	View Spectrum
1D NMR	¹H NMR Spectrum (1D, D₂O, experimental)	2016-10-22	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, D₂O, experimental)	2016-10-22	View Spectrum
2D NMR	[¹H, ¹³C] NMR Spectrum (2D, 600 MHz, H₂O, predicted)	2012-12-04	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm

Biospecimen Locations

Blood
Breast Milk
Cerebrospinal Fluid (CSF)
Saliva
Sweat
Urine

Tissue Locations

Prostate

Pathways

Name	SMPDB/Pathwhiz	KEGG
Citric Acid Cycle
Congenital lactic acidosis		Not Available
Fumarase deficiency		Not Available
Mitochondrial complex II deficiency		Not Available
2-ketoglutarate dehydrogenase complex deficiency		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Breast Milk	Detected and Quantified	10.1 +/- 3.1 uM	Adult (>18 years old)	Female	Normal	24027187	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	2 uM	Children (1-13 years old)	Both	Normal	8412012	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	20.0 (13.0-27.0) uM	Adult (>18 years old)	Both	Normal	9693263	details
Saliva	Detected and Quantified	3.17 +/- 1.17 uM	Adult (>18 years old)	Female	Normal	23857558	details
Saliva	Detected and Quantified	0.172 +/- 0.179 uM	Adult (>18 years old)	Male	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	0.176 +/- 0.107 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	0.358 +/- 0.198 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	0.363 +/- 0.169 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Sweat	Detected and Quantified	< 10 uM	Adult (60 years old)	Male	Normal	22194922	details
Sweat	Detected and Quantified	< 10 uM	Adult (40 years old)	Male	Normal	22194922	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22932811	details
Urine	Detected and Quantified	67.9 (14.3-100.7) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	8087979	details
Urine	Detected and Quantified	73.8 (64.0-130.3) umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	8087979	details
Urine	Detected and Quantified	37.9 (17.3-63.3) umol/mmol creatinine	Children (1-13 years old)	Both	Normal	8087979	details
Urine	Detected and Quantified	29.8 (14.7-93.1) umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	8087979	details
Urine	Detected and Quantified	2.0-23.6 umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	31963255	details
Urine	Detected and Quantified	7-30.6 umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	31963255	details
Urine	Detected and Quantified	8-26.0 umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	31963255	details
Urine	Detected and Quantified	8-40.7 umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	31963255	details
Urine	Detected and Quantified	54.5 (32.4-76.6) umol/mmol creatinine	Children (1-13 years old)	Both	Normal	14708889	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	28157703	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21389975	details
Urine	Detected and Quantified	48-103 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected and Quantified	20.9 (3.8-95.3) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	13 (2.7-44) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	2702744	details
Urine	Detected and Quantified	9.740-38.962 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	12376931	details
Urine	Detected and Quantified	35.95 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	Shaykhutdinov RA,...	details
Urine	Detected and Quantified	<102.12 umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	58-85 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected and Quantified	33.685 +/- 14.03 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Mordechai, Hien, ...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	22024767	details
Urine	Detected and Quantified	10.3(5.2-16.3) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Saliva	Detected and Quantified	2.80 +/- 2.11 uM	Adult (>18 years old)	Male	Alzheimer's disease	23857558	details
Saliva	Detected and Quantified	2.17 +/- 0.53 uM	Adult (>18 years old)	Male	Frontotemporal lobe dementia	23857558	details
Saliva	Detected and Quantified	2.72 +/- 0.53 uM	Adult (>18 years old)	Both	Lewy body disease	23857558	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Bladder cancer	28157703	details
Urine	Detected and Quantified	6.494-293.514 umol/mmol creatinine	Infant (0-1 year old)	Both	Amish lethal microcephaly	12376931	details
Urine	Detected and Quantified	38.74 +/- 40.952 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Mordechai, Hien, ...	details
Urine	Detected and Quantified	32.0 (0.0-70.0) umol/mmol creatinine	Adult (>18 years old)	Both	Lung cancer	18953024	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Schizophrenia	22024767	details

Associated Disorders and Diseases

Disease References

Alzheimer's disease
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Frontotemporal dementia
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Lewy body disease
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Lung Cancer
Wishart DS, Knox C, Guo AC, Eisner R, Young N, Gautam B, Hau DD, Psychogios N, Dong E, Bouatra S, Mandal R, Sinelnikov I, Xia J, Jia L, Cruz JA, Lim E, Sobsey CA, Shrivastava S, Huang P, Liu P, Fang L, Peng J, Fradette R, Cheng D, Tzur D, Clements M, Lewis A, De Souza A, Zuniga A, Dawe M, Xiong Y, Clive D, Greiner R, Nazyrova A, Shaykhutdinov R, Li L, Vogel HJ, Forsythe I: HMDB: a knowledgebase for the human metabolome. Nucleic Acids Res. 2009 Jan;37(Database issue):D603-10. doi: 10.1093/nar/gkn810. Epub 2008 Oct 25. [PubMed:18953024 ]
Eosinophilic esophagitis
(). Mordechai, Hien, and David S. Wishart. .
Schizophrenia
Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767 ]
Amish lethal microcephaly
Kelley RI, Robinson D, Puffenberger EG, Strauss KA, Morton DH: Amish lethal microcephaly: a new metabolic disorder with severe congenital microcephaly and 2-ketoglutaric aciduria. Am J Med Genet. 2002 Nov 1;112(4):318-26. doi: 10.1002/ajmg.10529. [PubMed:12376931 ]

Associated OMIM IDs

104300 (Alzheimer's disease)
600274 (Frontotemporal dementia)
211980 (Lung Cancer)
610247 (Eosinophilic esophagitis)
181500 (Schizophrenia)
607196 (Amish lethal microcephaly)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Not Available

Wikipedia Link

Citric acid cycle

METLIN ID

5130

PubChem Compound

643757

PDB ID

Not Available

ChEBI ID

32805

Food Biomarker Ontology

Not Available

VMH ID

Not Available

MarkerDB ID

MDB00000038

References

Synthesis Reference

Gutierrez, Eddie N.; Lamberti, Vincent. cis and trans Aconitic acids and their salts. U.S. (1978), 16 pp.

Material Safety Data Sheet (MSDS)

Download (PDF)

General References

Swart PJ, Kuipers EM, Smit C, Van Der Strate BW, Harmsen MC, Meijer DK: Lactoferrin. Antiviral activity of lactoferrin. Adv Exp Med Biol. 1998;443:205-13. [PubMed:9781360 ]
Stromme JH, Borud O, Moe PJ: Fatal lactic acidosis in a newborn attributable to a congenital defect of pyruvate dehydrogenase. Pediatr Res. 1976 Jan;10(1):62-6. [PubMed:813176 ]
Boulat O, Gradwohl M, Matos V, Guignard JP, Bachmann C: Organic acids in the second morning urine in a healthy Swiss paediatric population. Clin Chem Lab Med. 2003 Dec;41(12):1642-58. [PubMed:14708889 ]
Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed:8087979 ]
Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. [PubMed:9693263 ]
Swart PJ, Kuipers ME, Smit C, Pauwels R, deBethune MP, de Clercq E, Meijer DK, Huisman JG: Antiviral effects of milk proteins: acylation results in polyanionic compounds with potent activity against human immunodeficiency virus types 1 and 2 in vitro. AIDS Res Hum Retroviruses. 1996 Jun 10;12(9):769-75. [PubMed:8738428 ]
Matsuishi T, Urabe F, Percy AK, Komori H, Yamashita Y, Schultz RS, Ohtani Y, Kuriya N, Kato H: Abnormal carbohydrate metabolism in cerebrospinal fluid in Rett syndrome. J Child Neurol. 1994 Jan;9(1):26-30. [PubMed:8151077 ]
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 ]

Enzymes

Enzyme Details

1. Aconitate hydratase, mitochondrial

General function:: Involved in metabolic process
Specific function:: Catalyzes the isomerization of citrate to isocitrate via cis-aconitate (By similarity).
Gene Name:: ACO2
Uniprot ID:: Q99798
Molecular weight:: 85424.745

Reactions

Citric acid → cis-Aconitic acid + Water	details
Isocitric acid → cis-Aconitic acid + Water	details

Enzyme Details

2. Cytoplasmic aconitate hydratase

General function:: Involved in metabolic process
Specific function:: Iron sensor. Binds a 4Fe-4S cluster and functions as aconitase when cellular iron levels are high. Functions as mRNA binding protein that regulates uptake, sequestration and utilization of iron when cellular iron levels are low. Binds to iron-responsive elements (IRES) in target mRNA species when iron levels are low. Binding of a 4Fe-4S cluster precludes RNA binding. Catalyzes the isomerization of citrate to isocitrate via cis-aconitate (By similarity).
Gene Name:: ACO1
Uniprot ID:: P21399
Molecular weight:: 98398.14

Reactions

Citric acid → cis-Aconitic acid + Water	details
Isocitric acid → cis-Aconitic acid + Water	details

Showing metabocard for cis-Aconitic acid (HMDB0000072)

MOL for HMDB0000072 (cis-Aconitic acid)

3D MOL for HMDB0000072 (cis-Aconitic acid)

3D SDF for HMDB0000072 (cis-Aconitic acid)

3D-SDF for HMDB0000072 (cis-Aconitic acid)

PDB for HMDB0000072 (cis-Aconitic acid)

3D PDB for HMDB0000072 (cis-Aconitic acid)

SMILES for HMDB0000072 (cis-Aconitic acid)

INCHI for HMDB0000072 (cis-Aconitic acid)

Structure for HMDB0000072 (cis-Aconitic acid)

3D Structure for HMDB0000072 (cis-Aconitic acid)

GC-MS

LC-MS/MS

NMR

Enzymes

Reactions

Reactions