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Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2023-05-30 20:55:55 UTC

HMDB ID

HMDB0000254

Secondary Accession Numbers

HMDB00254

Metabolite Identification

Common Name

Succinic acid

Description

Succinic acid (succinate) is a dicarboxylic acid. It is an important component of the citric acid or TCA cycle and is capable of donating electrons to the electron transfer chain. Succinate is found in all living organisms ranging from bacteria to plants to mammals. In eukaryotes, succinate is generated in the mitochondria via the tricarboxylic acid cycle (TCA). Succinate can readily be imported into the mitochondrial matrix by the n-butylmalonate- (or phenylsuccinate-) sensitive dicarboxylate carrier in exchange with inorganic phosphate or another organic acid, e. g. malate (PMID 16143825 ). Succinate can exit the mitochondrial matrix and function in the cytoplasm as well as the extracellular space. Succinate has multiple biological roles including roles as a metabolic intermediate and roles as a cell signalling molecule. Succinate can alter gene expression patterns, thereby modulating the epigenetic landscape or it can exhibit hormone-like signaling functions (PMID: 26971832 ). As such, succinate links cellular metabolism, especially ATP formation, to the regulation of cellular function. Succinate can be broken down or metabolized into fumarate by the enzyme succinate dehydrogenase (SDH), which is part of the electron transport chain involved in making ATP. Dysregulation of succinate synthesis, and therefore ATP synthesis, can happen in a number of genetic mitochondrial diseases, such as Leigh syndrome, and Melas syndrome. Succinate has been found to be associated with D-2-hydroxyglutaric aciduria, which is an inborn error of metabolism. Succinic acid has recently been identified as an oncometabolite or an endogenous, cancer causing metabolite. High levels of this organic acid can be found in tumors or biofluids surrounding tumors. Its oncogenic action appears to due to its ability to inhibit prolyl hydroxylase-containing enzymes. In many tumours, oxygen availability becomes limited (hypoxia) very quickly due to rapid cell proliferation and limited blood vessel growth. The major regulator of the response to hypoxia is the HIF transcription factor (HIF-alpha). Under normal oxygen levels, protein levels of HIF-alpha are very low due to constant degradation, mediated by a series of post-translational modification events catalyzed by the prolyl hydroxylase domain-containing enzymes PHD1, 2 and 3, (also known as EglN2, 1 and 3) that hydroxylate HIF-alpha and lead to its degradation. All three of the PHD enzymes are inhibited by succinate. In humans, urinary succinic acid is produced by Escherichia coli, Pseudomonas aeruginosa, Klebsiella pneumonia, Enterobacter, Acinetobacter, Proteus mirabilis, Citrobacter frundii, Enterococcus faecalis (PMID: 22292465 ). Succinic acid is also found in Actinobacillus, Anaerobiospirillum, Mannheimia, Corynebacterium and Basfia (PMID: 22292465 ; PMID: 18191255 ; PMID: 26360870 ).

Structure

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

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
1,2-Ethanedicarboxylic acid	ChEBI
Acide butanedioique	ChEBI
Acide succinique	ChEBI
Acidum succinicum	ChEBI
Amber acid	ChEBI
Asuccin	ChEBI
Bernsteinsaeure	ChEBI
Butandisaeure	ChEBI
Butanedionic acid	ChEBI
Dihydrofumaric acid	ChEBI
e363	ChEBI
Ethylenesuccinic acid	ChEBI
HOOC-CH2-CH2-COOH	ChEBI
Spirit OF amber	ChEBI
1,2-Ethanedicarboxylate	Generator
Butanedionate	Generator
Dihydrofumarate	Generator
Ethylenesuccinate	Generator
Succinate	Generator
1,2 Ethanedicarboxylic acid	MeSH
1,4 Butanedioic acid	MeSH
1,4-Butanedioic acid	MeSH
Ammonium succinate	MeSH
Butanedioic acid	MeSH
Potassium succinate	MeSH
Succinate, ammonium	MeSH
Succinate, potassium	MeSH
1,4-Butanedioate	HMDB
Katasuccin	HMDB
Wormwood acid	HMDB
2-Acetamido-2-deoxy-D-glucose	ChEBI, HMDB
D-GlcNAc	ChEBI, HMDB
N-Acetyl-D-glucosamine	ChEBI, HMDB
N-Acetylchitosamine	ChEBI, HMDB
N Acetyl D glucosamine	HMDB
2 Acetamido 2 deoxy D glucose	HMDB
2 Acetamido 2 deoxyglucose	HMDB
2-Acetamido-2-deoxyglucose	HMDB
Acetylglucosamine	HMDB

Chemical Formula

C₄H₆O₄

Average Molecular Weight

118.088

Monoisotopic Molecular Weight

118.02660868

IUPAC Name

butanedioic acid

Traditional Name

succinic acid

CAS Registry Number

110-15-6

SMILES

OC(=O)CCC(O)=O

InChI Identifier

InChI=1S/C4H6O4/c5-3(6)1-2-4(7)8/h1-2H2,(H,5,6)(H,7,8)

InChI Key

KDYFGRWQOYBRFD-UHFFFAOYSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as dicarboxylic acids and derivatives. These are organic compounds containing exactly two carboxylic acid groups.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Carboxylic acids and derivatives

Sub Class

Dicarboxylic acids and derivatives

Direct Parent

Dicarboxylic acids and derivatives

Alternative Parents

Substituents

Fatty acid
Dicarboxylic 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

C4-dicarboxylic acid (CHEBI:15741 )
alpha,omega-dicarboxylic acid (CHEBI:15741 )
Dicarboxylic acids (LMFA01170043 )

Ontology

Not Available

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	185 - 188 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	83.2 mg/mL	Not Available
LogP	-0.59	HANSCH,C ET AL. (1995)

Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct Type	Data Source	CCS Value (Å²)	Reference
[M-H]-	Baker	119.841	30932474
[M+H]+	Baker	122.656	30932474
[M-H]-	Not Available	118.0	http://allccs.zhulab.cn/database/detail?ID=AllCCS00001762
[M+H]+	Not Available	122.656	http://allccs.zhulab.cn/database/detail?ID=AllCCS00001762

Predicted Molecular Properties

Property	Value	Source
Water Solubility	211 g/L	ALOGPS
logP	-0.53	ALOGPS
logP	-0.4	ChemAxon
logS	0.25	ALOGPS
pKa (Strongest Acidic)	3.55	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	23.54 m³·mol⁻¹	ChemAxon
Polarizability	10.14 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]+	124.478	31661259
DarkChem	[M-H]-	117.776	31661259
AllCCS	[M+H]+	128.067	32859911
AllCCS	[M-H]-	123.254	32859911
DeepCCS	[M+H]+	123.013	30932474
DeepCCS	[M-H]-	120.237	30932474
DeepCCS	[M-2H]-	156.607	30932474
DeepCCS	[M+Na]+	131.534	30932474
AllCCS	[M+H]+	128.1	32859911
AllCCS	[M+H-H2O]+	123.8	32859911
AllCCS	[M+NH4]+	132.0	32859911
AllCCS	[M+Na]+	133.1	32859911
AllCCS	[M-H]-	123.3	32859911
AllCCS	[M+Na-2H]-	126.4	32859911
AllCCS	[M+HCOO]-	129.9	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
Succinic acid	OC(=O)CCC(O)=O	1925.6	Standard polar	33892256
Succinic acid	OC(=O)CCC(O)=O	853.5	Standard non polar	33892256
Succinic acid	OC(=O)CCC(O)=O	1214.2	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
Succinic acid,1TMS,isomer #1	C[Si](C)(C)OC(=O)CCC(=O)O	1238.9	Semi standard non polar	33892256
Succinic acid,2TMS,isomer #1	C[Si](C)(C)OC(=O)CCC(=O)O[Si](C)(C)C	1321.3	Semi standard non polar	33892256
Succinic acid,1TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CCC(=O)O	1497.1	Semi standard non polar	33892256
Succinic acid,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OC(=O)CCC(=O)O[Si](C)(C)C(C)(C)C	1743.0	Semi standard non polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental GC-MS	GC-MS Spectrum - Succinic acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-0002-0920000000-f286e6204a4163b823ba	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Succinic acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-0002-0900000000-bf336910bb37d7f78140	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Succinic acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-006t-9800000000-df5ff4e8457d2d4ef919	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Succinic acid GC-MS (2 TMS)	splash10-00c1-3930000000-3cc18e719822b5af661a	2014-06-16	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Succinic acid EI-B (Non-derivatized)	splash10-004i-9000000000-93b4807ae6275a3e59d7	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Succinic acid EI-B (Non-derivatized)	splash10-0kos-9100000000-f1df0903a24c305e68ec	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Succinic acid EI-B (Non-derivatized)	splash10-0002-0910000000-300c33b39fb991b5a73e	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Succinic acid GC-EI-TOF (Non-derivatized)	splash10-0002-0920000000-f286e6204a4163b823ba	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Succinic acid GC-EI-TOF (Non-derivatized)	splash10-0002-0900000000-bf336910bb37d7f78140	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Succinic acid GC-EI-TOF (Non-derivatized)	splash10-006t-9800000000-df5ff4e8457d2d4ef919	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Succinic acid GC-MS (Non-derivatized)	splash10-00c1-3930000000-3cc18e719822b5af661a	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - Succinic acid GC-EI-TOF (Non-derivatized)	splash10-0002-0900000000-f838d863ee7c2b111f02	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Succinic acid GC-MS (Non-derivatized) - 70eV, Positive	splash10-00xr-9300000000-f0644daf4fbb11fcc2dc	2016-09-22	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Succinic acid GC-MS (2 TMS) - 70eV, Positive	splash10-00dj-9710000000-ff8325384b9eefd19106	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Succinic acid GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Succinic acid GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Succinic acid GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Succinic acid GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Succinic acid GC-MS (TBDMS_2_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
MS	Mass Spectrum (Electron Ionization)	splash10-05di-9100000000-c629bea41d0d3d896425	2014-09-20	Not Available	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid Quattro_QQQ 10V, Negative-QTOF (Annotated)	splash10-00di-9300000000-f9dc864d93a09d3074f9	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid Quattro_QQQ 25V, Negative-QTOF (Annotated)	splash10-00di-9300000000-76c151de384928b2256f	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid Quattro_QQQ 40V, Negative-QTOF (Annotated)	splash10-01b9-7900000000-51d2341c097f04827944	2012-07-24	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid EI-B (Unknown) , Positive-QTOF	splash10-004i-9000000000-93b4807ae6275a3e59d7	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative-QTOF	splash10-03dj-0971010000-37d214dc7a8fdc26116b	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative-QTOF	splash10-014i-9000000000-249222ac742c1634cec9	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative-QTOF	splash10-00di-9000000000-6897d49472dba6a34a27	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-ITFT (LTQ Orbitrap XL, Thermo Scientfic) , Negative-QTOF	splash10-0udi-0490000000-d138f8023125921b4b82	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative-QTOF	splash10-014i-1900000000-4ffdabe5bde527b66982	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative-QTOF	splash10-00di-9100000000-c20baa818f5ff5f678c1	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative-QTOF	splash10-00di-9000000000-7a49a18aa6fcb2540a12	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative-QTOF	splash10-00di-9000000000-9955aeb0e5a9f88ae70e	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negative-QTOF	splash10-00di-9000000000-7e1f195f111b4eafb4fa	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative-QTOF	splash10-00xr-9400000000-e50afc90e20cd420ba9b	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative-QTOF	splash10-00xr-9600000000-43167f2549cbb5d5f7e8	2012-08-31	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-QQ , negative-QTOF	splash10-014i-1900000000-4ffdabe5bde527b66982	2017-09-14	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-QQ , negative-QTOF	splash10-00di-9100000000-7a8bfa543dc087bea06d	2017-09-14	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-QQ , negative-QTOF	splash10-00di-9000000000-fdec6c7458176f3cbeb8	2017-09-14	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Succinic acid LC-ESI-QQ , negative-QTOF	splash10-00di-9000000000-9955aeb0e5a9f88ae70e	2017-09-14	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Succinic acid 10V, Positive-QTOF	splash10-0gb9-2900000000-9d959a53833b07094158	2016-09-12	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Succinic acid 20V, Positive-QTOF	splash10-0l6r-9600000000-c367e11e737714d41418	2016-09-12	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Succinic acid 40V, Positive-QTOF	splash10-056r-9000000000-e65aa602a8293debec36	2016-09-12	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Succinic acid 10V, Negative-QTOF	splash10-014i-2900000000-e1f840494c9003279869	2016-09-12	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Succinic acid 20V, Negative-QTOF	splash10-014i-7900000000-1089efd4a3469bcf14f1	2016-09-12	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Succinic acid 40V, Negative-QTOF	splash10-0604-9000000000-0e0e60bb202ffb004894	2016-09-12	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Experimental 1D NMR	¹H NMR Spectrum (1D, 500 MHz, H₂O, experimental)	2012-12-04	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Experimental 2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)	2012-12-05	Wishart Lab	View Spectrum

IR Spectra

Spectrum Type	Description	Deposition Date	Source	View
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
Mitochondria
Endoplasmic reticulum
Peroxisome

Biospecimen Locations

Blood
Breast Milk
Cerebrospinal Fluid (CSF)
Feces
Saliva
Sweat
Urine

Tissue Locations

Adipose Tissue
Brain
Fibroblasts
Kidney
Liver
Pancreas
Placenta
Prostate
Skeletal Muscle
Spleen

Pathways

Name	SMPDB/PathBank	KEGG
Citric Acid Cycle
Glutamate Metabolism
Oxidative phosphorylation	Not Available
Phytanic Acid Peroxisomal Oxidation		Not Available
Carnitine Synthesis		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	16.0 (0.00-32.0) uM	Adult (>18 years old)	Both	Normal	11067816	details
Blood	Detected and Quantified	23.5 uM	Adult (>18 years old)	Both	Normal	21359215	details
Blood	Detected and Quantified	8.8 +/- 2.7 uM	Adult (>18 years old)	Both	Normal	Geigy Scientific ...	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Blood	Detected and Quantified	6.10-11.5 uM	Adult (>18 years old)	Both	Normal	Geigy Scientific ...	details
Breast Milk	Detected and Quantified	8.4 +/- 2.9 uM	Adult (>18 years old)	Female	Normal	24027187	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	47.2 uM	Adult (>18 years old)	Both	Normal	18953024	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	19 uM	Adult (>18 years old)	Both	Normal	7729054	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	150.0 +/- 110.0 uM	Elderly (>65 years old)	Both	Normal	9693263	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	3 +/- 2 uM	Adult (>18 years old)	Both	Normal	18502700	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	28.5 (24-33) uM	Adult (>18 years old)	Both	Normal	Geigy Scientific ...	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	3.0 +/- 2.0 uM	Adult (>18 years old)	Both	Normal	8412012	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	28270806	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25670064	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	20669995	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	29808030	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Normal	Mette S. Sch...	details
Feces	Detected but not Quantified	Not Quantified	Infant (0-1 year old)	Both	Normal	22411374	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Normal	25985090	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	22944170	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Normal	Christopher ...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	18052033	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	19573517	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21761941	details
Feces	Detected and Quantified	132.528 +/- 151.836 nmol/g wet feces	Not Specified	Not Specified	Normal	19573517	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	18085514	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27065191	details
Feces	Detected and Quantified	1633.528 +/- 1822.370 nmol/g wet feces	Not Specified	Not Specified	Normal	20669995	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27543620	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Normal	27609529	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27107423	details
Feces	Detected and Quantified	255 nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	26416813	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25037050	details
Feces	Detected and Quantified	222 nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	26416813	details
Feces	Detected and Quantified	250 nmol/g wet feces	Adult (>18 years old)	Not Specified	Normal	26416813	details
Saliva	Detected and Quantified	125.15 +/- 180.60 uM	Adult (>18 years old)	Both	Normal	Zerihun T. Dame, ...	details
Saliva	Detected and Quantified	2260 (60.0-4460) uM	Adult (>18 years old)	Both	Normal	12097436	details
Saliva	Detected and Quantified	14 (2-112) uM	Adult (>18 years old)	Male	Normal	19259987	details
Saliva	Detected and Quantified	9 (1-78) uM	Adult (>18 years old)	Male	Normal	19259987	details
Saliva	Detected and Quantified	21 (<1-66) uM	Adult (>18 years old)	Male	Normal	19259987	details
Saliva	Detected and Quantified	9 (<1-54) uM	Adult (>18 years old)	Female	Normal	19259987	details
Saliva	Detected and Quantified	9.03 +/- 5.22 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	1268322	details
Saliva	Detected and Quantified	15.0 +/- 15.5 uM	Adult (>18 years old)	Not Specified	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	15.6 +/- 27.7 uM	Adult (>18 years old)	Not Specified	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
Saliva	Detected and Quantified	24.3 +/- 20.4 uM	Adult (>18 years old)	Male	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	4-145 uM	Adult (>18 years old)	Male	normal	19259987	details
Saliva	Detected and Quantified	1-33 uM	Adult (>18 years old)	Male	normal	19259987	details
Sweat	Detected but not Quantified	Not Quantified	Adult	Both	Normal	26755145	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22932811	details
Urine	Detected and Quantified	4.7 (1.1-14.5) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	9.4 +/- 9.2 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	31775291	details
Urine	Detected and Quantified	4.35-81.2 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	32340350	details
Urine	Detected and Quantified	16.99(5.66-67.50) umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	32340350	details
Urine	Detected and Quantified	16.76(3.97-85.17) umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	32340350	details
Urine	Detected and Quantified	12.60 +/- 12.13 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	2026685	details
Urine	Detected and Quantified	14.48 +/- 3.20 umol/mmol creatinine	Children (1-13 years old)	Both	Normal	10731506	details
Urine	Detected and Quantified	<76 umol/mmol creatinine	Not Specified	Not Specified	Normal	1640293	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	7.7 (1.9-20.0) umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	8087979	details
Urine	Detected and Quantified	3.8 (1.25-6.7) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	Geigy Scientific ...	details
Urine	Detected and Quantified	11.9-24.1 umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	31963255	details
Urine	Detected and Quantified	15.7-69.5 umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	31963255	details
Urine	Detected and Quantified	20.2-49.6 umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	31963255	details
Urine	Detected and Quantified	22.0-42.0 umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	31963255	details
Urine	Detected and Quantified	110 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	25012580	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21389975	details
Urine	Detected and Quantified	1.4-2.3 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	28157703	details
Urine	Detected and Quantified	14.581 +/- 9.663 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected and Quantified	2.10 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	Shaykhutdinov RA,...	details
Urine	Detected and Quantified	<87.14 umol/mmol creatinine	Children (1 - 18 years old)	Both	Normal	BC Children's Hos...	details
Urine	Detected and Quantified	9.9 +/- 5.0 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	17474139	details
Urine	Detected and Quantified	14.4 +/- 4.9 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	17474139	details
Urine	Detected and Quantified	7.5 (0.5-16) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	2702744	details
Urine	Detected and Quantified	6.0 (0.3-33.3) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	111.02 (31.39-241.29) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Analysis of 40 NI...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	16939360	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	16939360	details
Urine	Detected and Quantified	5.6 +/- 3.8 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	15992788	details
Urine	Detected and Quantified	197.2 (29.4-486.2) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	8087979	details
Urine	Detected and Quantified	185.4 (6.0-342.6) umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	8087979	details
Urine	Detected and Quantified	11.6 (4.0-27.3) umol/mmol creatinine	Children (1-13 years old)	Both	Normal	8087979	details
Urine	Detected and Quantified	8.25 (0.5-16.0) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	11067816	details
Urine	Detected and Quantified	1.4-1.7 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected and Quantified	163.491 +/- 191.759 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected and Quantified	<335.0976 umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	26078636	details
Urine	Detected and Quantified	1.915-71.807 umol/mmol creatinine	Children (1 - 13 years old)	Male	Normal	6774165	details
Urine	Detected and Quantified	1.3 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	21388201	details
Urine	Detected and Quantified	<97 umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	24341803	details
Urine	Detected and Quantified	9-360 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	31506554	details
Urine	Detected and Quantified	6.2 (2.5-13.5) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	5.3 (5.6) uM	Adult (>18 years old)	Female	Down syndrome pregnancy	23313728	details
Blood	Detected and Quantified	5.6 (12.5) uM	Adult (>18 years old)	Female	Pregnancy	23313728	details
Blood	Detected and Quantified	6.90 +/- 6.33 uM	Adult (>18 years old)	Female	Pregnancy with fetuses with trisomy 18	23535240	details
Blood	Detected and Quantified	6.50 +/- 10.83 uM	Adult (>18 years old)	Female	Pregnancy	23535240	details
Blood	Detected and Quantified	4.3 (1.6) uM	Adult (>18 years old)	Female	Early preeclampsia	22494326	details
Blood	Detected and Quantified	12.4 (13.9) uM	Adult (>18 years old)	Female	Pregnancy	22494326	details
Blood	Detected and Quantified	3.35 (1.78) uM	Adult (>18 years old)	Female	Pregnancy with fetus having congenital heart defect	24704061	details
Blood	Detected and Quantified	3.31 (1.13) uM	Adult (>18 years old)	Female	Pregnancy	24704061	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	19.0 (0.0-38.0) uM	Adult (>18 years old)	Both	Canavan Disease	7729054	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	55.0 (19.0-91.0) uM	Adult (>18 years old)	Both	Alzheimer's disease	9693263	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Gout	28270806	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	27015276	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal Cancer	27275383	details
Feces	Detected but not Quantified	Not Quantified	Infant (0-1 year old)	Not Specified	Genetically At-Risk of celiac disease	22432018	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Cryptosporidium infection	22944170	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Myalgic encephalomyelitis/chronic fatigue syndrome	Christopher ...	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25037050	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Irritable bowel syndrome	21761941	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Ulcerative colitis	21761941	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Crohns disease	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Ulcerative colitis	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Unclassified IBD	27609529	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal Cancer	27107423	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Crohn's disease	17269711	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Attachment loss	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Missing teeth	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Periodontal Probing Depth	31026179	details
Urine	Detected and Quantified	749-784 umol/mmol creatinine	Infant (0-1 year old)	Male	D-2-hydroxyglutaric aciduria	11999977	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Epithelial ovarian cancer	23163809	details
Urine	Detected and Quantified	75-185 umol/mmol creatinine	Infant (0-1 year old)	Male	2-Ketoglutarate dehydrogenase complex deficiency	1640293	details
Urine	Detected and Quantified	43.35 +/- 25.08 umol/mmol creatinine	Children (1-13 years old)	Both	Malnutrition (type kwashiorkor and marasmus)	10731506	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Bladder cancer	28157703	details
Urine	Detected and Quantified	27.897 +/- 29.998 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected and Quantified	9.0 +/- 6.0 umol/mmol creatinine	Adult (>18 years old)	Both	Lung cancer	18953024	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Urine	Detected and Quantified	4.787 umol/mmol creatinine	Children (1 - 13 years old)	Male	D-2-Hydroxyglutaric aciduria	6774165	details
Urine	Detected and Quantified	467.612 +/- 705.456 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected and Quantified	6.667 umol/mmol creatinine	Children (1 - 13 years old)	Male	D-2-Hydroxyglutaric aciduria	6774165	details
Urine	Detected and Quantified	530.857 +/- 876.714 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Gastroesophageal reflux disease	Analysis of 30 no...	details
Urine	Detected and Quantified	50 umol/mmol creatinine	Infant (0-1 year old)	Male	Malonyl-Coa decarboxylase deficiency	3709568	details
Urine	Detected and Quantified	86.168 umol/mmol creatinine	Children (1 - 13 years old)	Male	D-2-Hydroxyglutaric aciduria	6774165	details
Urine	Detected and Quantified	141.698 umol/mmol creatinine	Infant (0-1 year old)	Male	Fumarase deficiency	26078636	details
Urine	Detected and Quantified	15 umol/mmol creatinine	Children (1-13 years old)	Male	Lipoyltransferase 1 Deficiency	24341803	details

Associated Disorders and Diseases

Disease References

Early preeclampsia
Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
Pregnancy
Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ] Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester Down syndrome prediction. Am J Obstet Gynecol. 2013 May;208(5):371.e1-8. doi: 10.1016/j.ajog.2012.12.035. Epub 2013 Jan 8. [PubMed:23313728 ] Bahado-Singh RO, Akolekar R, Chelliah A, Mandal R, Dong E, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester trisomy 18 detection. Am J Obstet Gynecol. 2013 Jul;209(1):65.e1-9. doi: 10.1016/j.ajog.2013.03.028. Epub 2013 Mar 25. [PubMed:23535240 ] Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061 ]
Canavan disease
Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. [PubMed:7729054 ]
Alzheimer's disease
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 ]
Irritable bowel syndrome
Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ]
Ulcerative colitis
Le Gall G, Noor SO, Ridgway K, Scovell L, Jamieson C, Johnson IT, Colquhoun IJ, Kemsley EK, Narbad A: Metabolomics of fecal extracts detects altered metabolic activity of gut microbiota in ulcerative colitis and irritable bowel syndrome. J Proteome Res. 2011 Sep 2;10(9):4208-18. doi: 10.1021/pr2003598. Epub 2011 Aug 8. [PubMed:21761941 ] Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Crohn's disease
Marchesi JR, Holmes E, Khan F, Kochhar S, Scanlan P, Shanahan F, Wilson ID, Wang Y: Rapid and noninvasive metabonomic characterization of inflammatory bowel disease. J Proteome Res. 2007 Feb;6(2):546-51. [PubMed:17269711 ] Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
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 ]
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 ]
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
Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Colorectal cancer
Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14. [PubMed:25105552 ] Lin Y, Ma C, Liu C, Wang Z, Yang J, Liu X, Shen Z, Wu R: NMR-based fecal metabolomics fingerprinting as predictors of earlier diagnosis in patients with colorectal cancer. Oncotarget. 2016 May 17;7(20):29454-64. doi: 10.18632/oncotarget.8762. [PubMed:27107423 ] 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 ]
D-2-hydroxyglutaric aciduria
Chalmers RA, Lawson AM, Watts RW, Tavill AS, Kamerling JP, Hey E, Ogilvie D: D-2-hydroxyglutaric aciduria: case report and biochemical studies. J Inherit Metab Dis. 1980;3(1):11-5. [PubMed:6774165 ] Wajne M, Vargas CR, Funayama C, Fernandez A, Elias ML, Goodman SI, Jakobs C, van der Knaap MS: D-2-Hydroxyglutaric aciduria in a patient with a severe clinical phenotype and unusual MRI findings. J Inherit Metab Dis. 2002 Feb;25(1):28-34. [PubMed:11999977 ]
Malonyl-Coa decarboxylase deficiency
Haan EA, Scholem RD, Croll HB, Brown GK: Malonyl coenzyme A decarboxylase deficiency. Clinical and biochemical findings in a second child with a more severe enzyme defect. Eur J Pediatr. 1986 Apr;144(6):567-70. [PubMed:3709568 ]
Fumarase deficiency
Bastug O, Kardas F, Ozturk MA, Halis H, Memur S, Korkmaz L, Tag Z, Gunes T: A rare cause of opistotonus; fumaric aciduria: The first case presentation in Turkey. Turk Pediatri Ars. 2014 Mar 1;49(1):74-6. doi: 10.5152/tpa.2014.442. eCollection 2014 Mar. [PubMed:26078636 ]
2-Ketoglutarate dehydrogenase complex deficiency
Bonnefont JP, Chretien D, Rustin P, Robinson B, Vassault A, Aupetit J, Charpentier C, Rabier D, Saudubray JM, Munnich A: Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis. J Pediatr. 1992 Aug;121(2):255-8. [PubMed:1640293 ]
Lipoyltransferase 1 Deficiency
Soreze Y, Boutron A, Habarou F, Barnerias C, Nonnenmacher L, Delpech H, Mamoune A, Chretien D, Hubert L, Bole-Feysot C, Nitschke P, Correia I, Sardet C, Boddaert N, Hamel Y, Delahodde A, Ottolenghi C, de Lonlay P: Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase. Orphanet J Rare Dis. 2013 Dec 17;8:192. doi: 10.1186/1750-1172-8-192. [PubMed:24341803 ]

Associated OMIM IDs

271900 (Canavan disease)
104300 (Alzheimer's disease)
266600 (Crohn's disease)
138900 (Gout)
211980 (Lung Cancer)
610247 (Eosinophilic esophagitis)
114500 (Colorectal cancer)
600721 (D-2-hydroxyglutaric aciduria)
248360 (Malonyl-Coa decarboxylase deficiency)
606812 (Fumarase deficiency)
203740 (2-Ketoglutarate dehydrogenase complex deficiency)
616299 (Lipoyltransferase 1 Deficiency)

External Links

DrugBank ID

DB00139

Phenol Explorer Compound ID

Not Available

FooDB ID

KNApSAcK ID

Chemspider ID

KEGG Compound ID

BioCyc ID

BiGG ID

Not Available

Wikipedia Link

Succinic_acid

METLIN ID

Not Available

PubChem Compound

1738118

PDB ID

Not Available

ChEBI ID

15741

Food Biomarker Ontology

Not Available

VMH ID

SUCC

MarkerDB ID

MDB00000124

Good Scents ID

Not Available

References

Synthesis Reference

Berglund, Kris Arvid; Andersson, Christian; Rova, Ulrika. Process for the production of succinic acid. PCT Int. Appl. (2007), 30pp.

Material Safety Data Sheet (MSDS)

Not Available

General References

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 ]
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 ]
Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. [PubMed:8412012 ]
Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. [PubMed:12097436 ]
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 ]
Wevers RA, Engelke U, Wendel U, de Jong JG, Gabreels FJ, Heerschap A: Standardized method for high-resolution 1H-NMR of cerebrospinal fluid. Clin Chem. 1995 May;41(5):744-51. [PubMed:7729054 ]
Magera MJ, Helgeson JK, Matern D, Rinaldo P: Methylmalonic acid measured in plasma and urine by stable-isotope dilution and electrospray tandem mass spectrometry. Clin Chem. 2000 Nov;46(11):1804-10. [PubMed:11067816 ]
Zhang TM, Sener A, Malaisse WJ: Hydrolysis of succinic acid dimethyl ester in rat pancreatic islets. Biochem Mol Med. 1995 Aug;55(2):131-7. [PubMed:7582870 ]
Groenen PM, Engelke UF, Wevers RA, Hendriks JC, Eskes TK, Merkus HM, Steegers-Theunissen RP: High-resolution 1H NMR spectroscopy of amniotic fluids from spina bifida fetuses and controls. Eur J Obstet Gynecol Reprod Biol. 2004 Jan 15;112(1):16-23. [PubMed:14687733 ]
Meijer-Severs GJ, van Santen E: Short-chain fatty acids and succinate in feces of healthy human volunteers and their correlation with anaerobe cultural counts. Scand J Gastroenterol. 1987 Aug;22(6):672-6. [PubMed:3659829 ]
Ren LC, Huang XY, Long JH: [Effects of succinic acid on the function of in vitro cultured human fibroblasts]. Zhonghua Shao Shang Za Zhi. 2004 Feb;20(1):34-6. [PubMed:15059451 ]
Wevers RA, Engelke U, Heerschap A: High-resolution 1H-NMR spectroscopy of blood plasma for metabolic studies. Clin Chem. 1994 Jul;40(7 Pt 1):1245-50. [PubMed:8013094 ]
Borenstein DG, Gibbs CA, Jacobs RP: Gas-liquid chromatographic analysis of synovial fluid: volatile short-chain fatty acids in septic arthritis. Ann Rheum Dis. 1983 Aug;42(4):362-7. [PubMed:6882030 ]
Frenkel G, Peterson RN, Freund M: Oxidative and glycolytic metabolism of semen components by washed guinea pig spermatozoa. Fertil Steril. 1975 Feb;26(2):144-7. [PubMed:1126459 ]
Briere JJ, Favier J, El Ghouzzi V, Djouadi F, Benit P, Gimenez AP, Rustin P: Succinate dehydrogenase deficiency in human. Cell Mol Life Sci. 2005 Oct;62(19-20):2317-24. [PubMed:16143825 ]
Rustin P, Rotig A: Inborn errors of complex II--unusual human mitochondrial diseases. Biochim Biophys Acta. 2002 Jan 17;1553(1-2):117-22. [PubMed:11803021 ]
Gupta A, Dwivedi M, Mahdi AA, Khetrapal CL, Bhandari M: Broad identification of bacterial type in urinary tract infection using (1)h NMR spectroscopy. J Proteome Res. 2012 Mar 2;11(3):1844-54. doi: 10.1021/pr2010692. Epub 2012 Jan 31. [PubMed:22292465 ]
Sauer M, Porro D, Mattanovich D, Branduardi P: Microbial production of organic acids: expanding the markets. Trends Biotechnol. 2008 Feb;26(2):100-8. doi: 10.1016/j.tibtech.2007.11.006. Epub 2008 Jan 11. [PubMed:18191255 ]
Becker J, Lange A, Fabarius J, Wittmann C: Top value platform chemicals: bio-based production of organic acids. Curr Opin Biotechnol. 2015 Dec;36:168-75. doi: 10.1016/j.copbio.2015.08.022. Epub 2015 Sep 8. [PubMed:26360870 ]
Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]
Tretter L, Patocs A, Chinopoulos C: Succinate, an intermediate in metabolism, signal transduction, ROS, hypoxia, and tumorigenesis. Biochim Biophys Acta. 2016 Aug;1857(8):1086-1101. doi: 10.1016/j.bbabio.2016.03.012. Epub 2016 Mar 10. [PubMed:26971832 ]

Only showing the first 10 proteins. There are 44 proteins in total.

Show all enzymes and transporters

Enzymes

Enzyme Details

1. Succinate dehydrogenase cytochrome b560 subunit, mitochondrial

General function:: Involved in succinate dehydrogenase activity
Specific function:: Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).
Gene Name:: SDHC
Uniprot ID:: Q99643
Molecular weight:: 16650.185

Reactions

Ubiquinone-2 + Succinic acid → QH2 + Fumaric acid	details

References

Bayley JP, van Minderhout I, Weiss MM, Jansen JC, Oomen PH, Menko FH, Pasini B, Ferrando B, Wong N, Alpert LC, Williams R, Blair E, Devilee P, Taschner PE: Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma. BMC Med Genet. 2006 Jan 11;7:1. [PubMed:16405730 ]
Leibowitz G, Khaldi MZ, Shauer A, Parnes M, Oprescu AI, Cerasi E, Jonas JC, Kaiser N: Mitochondrial regulation of insulin production in rat pancreatic islets. Diabetologia. 2005 Aug;48(8):1549-59. Epub 2005 Jun 29. [PubMed:15986240 ]
Bayley JP, Devilee P, Taschner PE: The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency. BMC Med Genet. 2005 Nov 16;6:39. [PubMed:16288654 ]
Szeto SS, Reinke SN, Sykes BD, Lemire BD: Ubiquinone-binding site mutations in the Saccharomyces cerevisiae succinate dehydrogenase generate superoxide and lead to the accumulation of succinate. J Biol Chem. 2007 Sep 14;282(37):27518-26. Epub 2007 Jul 18. [PubMed:17636259 ]
Kubo Y, Takagi H, Nakamori S: Effect of gene disruption of succinate dehydrogenase on succinate production in a sake yeast strain. J Biosci Bioeng. 2000;90(6):619-24. [PubMed:16232921 ]

Enzyme Details

2. Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial

General function:: Involved in electron carrier activity
Specific function:: Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Can act as a tumor suppressor.
Gene Name:: SDHA
Uniprot ID:: P31040
Molecular weight:: 72690.975

Reactions

Succinic acid + Coenzyme Q10 → Fumaric acid + QH(2)	details
Ubiquinone-2 + Succinic acid → QH2 + Fumaric acid	details

References

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
Maklashina E, Iverson TM, Sher Y, Kotlyar V, Andrell J, Mirza O, Hudson JM, Armstrong FA, Rothery RA, Weiner JH, Cecchini G: Fumarate reductase and succinate oxidase activity of Escherichia coli complex II homologs are perturbed differently by mutation of the flavin binding domain. J Biol Chem. 2006 Apr 21;281(16):11357-65. Epub 2006 Feb 15. [PubMed:16484232 ]
Takeo S, Kokaze A, Ng CS, Mizuchi D, Watanabe JI, Tanabe K, Kojima S, Kita K: Succinate dehydrogenase in Plasmodium falciparum mitochondria: molecular characterization of the SDHA and SDHB genes for the catalytic subunits, the flavoprotein (Fp) and iron-sulfur (Ip) subunits. Mol Biochem Parasitol. 2000 Apr 15;107(2):191-205. [PubMed:10779596 ]
Ackrell BA: Cytopathies involving mitochondrial complex II. Mol Aspects Med. 2002 Oct;23(5):369-84. [PubMed:12231007 ]

Enzyme Details

3. Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial

General function:: Involved in iron ion binding
Specific function:: Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (By similarity).
Gene Name:: SDHD
Uniprot ID:: O14521
Molecular weight:: 17042.82

Reactions

Ubiquinone-2 + Succinic acid → QH2 + Fumaric acid	details

References

Brink I, Schaefer O, Walz M, Neumann HP: Fluorine-18 DOPA PET imaging of paraganglioma syndrome. Clin Nucl Med. 2006 Jan;31(1):39-41. [PubMed:16374125 ]
Sun F, Huo X, Zhai Y, Wang A, Xu J, Su D, Bartlam M, Rao Z: Crystal structure of mitochondrial respiratory membrane protein complex II. Cell. 2005 Jul 1;121(7):1043-57. [PubMed:15989954 ]
Bayley JP, Devilee P, Taschner PE: The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency. BMC Med Genet. 2005 Nov 16;6:39. [PubMed:16288654 ]
Lehtonen HJ, Makinen MJ, Kiuru M, Laiho P, Herva R, van Minderhout I, Hogendoorn PC, Cornelisse C, Devilee P, Launonen V, Aaltonen LA: Increased HIF1 alpha in SDH and FH deficient tumors does not cause microsatellite instability. Int J Cancer. 2007 Sep 15;121(6):1386-9. [PubMed:17520677 ]
Bayley JP, van Minderhout I, Weiss MM, Jansen JC, Oomen PH, Menko FH, Pasini B, Ferrando B, Wong N, Alpert LC, Williams R, Blair E, Devilee P, Taschner PE: Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma. BMC Med Genet. 2006 Jan 11;7:1. [PubMed:16405730 ]

Enzyme Details

4. Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial

General function:: Involved in electron carrier activity
Specific function:: Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).
Gene Name:: SDHB
Uniprot ID:: P21912
Molecular weight:: 31629.365

Reactions

Succinic acid + Coenzyme Q10 → Fumaric acid + QH(2)	details
Ubiquinone-2 + Succinic acid → QH2 + Fumaric acid	details

References

Arikawa Y, Kuroyanagi T, Shimosaka M, Muratsubaki H, Enomoto K, Kodaira R, Okazaki M: Effect of gene disruptions of the TCA cycle on production of succinic acid in Saccharomyces cerevisiae. J Biosci Bioeng. 1999;87(1):28-36. [PubMed:16232421 ]
Bayley JP, Devilee P, Taschner PE: The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency. BMC Med Genet. 2005 Nov 16;6:39. [PubMed:16288654 ]
Lehtonen HJ, Makinen MJ, Kiuru M, Laiho P, Herva R, van Minderhout I, Hogendoorn PC, Cornelisse C, Devilee P, Launonen V, Aaltonen LA: Increased HIF1 alpha in SDH and FH deficient tumors does not cause microsatellite instability. Int J Cancer. 2007 Sep 15;121(6):1386-9. [PubMed:17520677 ]
Bayley JP, van Minderhout I, Weiss MM, Jansen JC, Oomen PH, Menko FH, Pasini B, Ferrando B, Wong N, Alpert LC, Williams R, Blair E, Devilee P, Taschner PE: Mutation analysis of SDHB and SDHC: novel germline mutations in sporadic head and neck paraganglioma and familial paraganglioma and/or pheochromocytoma. BMC Med Genet. 2006 Jan 11;7:1. [PubMed:16405730 ]
Szeto SS, Reinke SN, Sykes BD, Lemire BD: Ubiquinone-binding site mutations in the Saccharomyces cerevisiae succinate dehydrogenase generate superoxide and lead to the accumulation of succinate. J Biol Chem. 2007 Sep 14;282(37):27518-26. Epub 2007 Jul 18. [PubMed:17636259 ]

Enzyme Details

5. Succinate-semialdehyde dehydrogenase, mitochondrial

General function:: Involved in oxidoreductase activity
Specific function:: Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA).
Gene Name:: ALDH5A1
Uniprot ID:: P51649
Molecular weight:: 57214.23

Reactions

Succinic acid semialdehyde + NAD + Water → Succinic acid + NADH	details
Succinic acid semialdehyde + NAD + Water → Succinic acid + NADH + Hydrogen Ion	details

References

Yogeeswari P, Sriram D, Vaigundaragavendran J: The GABA shunt: an attractive and potential therapeutic target in the treatment of epileptic disorders. Curr Drug Metab. 2005 Apr;6(2):127-39. [PubMed:15853764 ]
Popov VN, Eprintsev AT, Fedorin DN, Fomenko OIu, Igamberdiev AU: [Role of transamination in the mobilization of respiratory substrates in germinating seeds of castor oil plants]. Prikl Biokhim Mikrobiol. 2007 May-Jun;43(3):376-81. [PubMed:17619587 ]
Wang C, Zhang HB, Wang LH, Zhang LH: Succinic semialdehyde couples stress response to quorum-sensing signal decay in Agrobacterium tumefaciens. Mol Microbiol. 2006 Oct;62(1):45-56. Epub 2006 Aug 30. [PubMed:16942602 ]
Ahn SJ, Yang CH, Cooksey DA: Pseudomonas putida 06909 genes expressed during colonization on mycelial surfaces and phenotypic characterization of mutants. J Appl Microbiol. 2007 Jul;103(1):120-32. [PubMed:17584458 ]
Chiribau CB, Mihasan M, Ganas P, Igloi GL, Artenie V, Brandsch R: Final steps in the catabolism of nicotine. FEBS J. 2006 Apr;273(7):1528-36. [PubMed:16689938 ]
Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [PubMed:11752352 ]

Enzyme Details

6. Phytanoyl-CoA dioxygenase, peroxisomal

General function:: Involved in electron carrier activity
Specific function:: Converts phytanoyl-CoA to 2-hydroxyphytanoyl-CoA.
Gene Name:: PHYH
Uniprot ID:: O14832
Molecular weight:: 38538.065

Reactions

Phytanoyl-CoA + Oxoglutaric acid + Oxygen → 2-Hydroxyphytanoyl-CoA + Succinic acid + CO(2)	details

Enzyme Details

7. Prolyl 4-hydroxylase subunit alpha-2

General function:: Involved in oxidoreductase activity
Specific function:: Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Gene Name:: P4HA2
Uniprot ID:: O15460
Molecular weight:: 60632.19

Reactions

L-proline-[procollagen] + Oxoglutaric acid + Oxygen → trans-4-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2)	details
Proline + Oxoglutaric acid + Oxygen → 4-Hydroxyproline + Succinic acid + Carbon dioxide	details

References

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]

Enzyme Details

8. Prolyl 4-hydroxylase subunit alpha-1

General function:: Involved in oxidoreductase activity
Specific function:: Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Gene Name:: P4HA1
Uniprot ID:: P13674
Molecular weight:: 60966.645

Reactions

L-proline-[procollagen] + Oxoglutaric acid + Oxygen → trans-4-hydroxy-L-proline-[procollagen] + Succinic acid + CO(2)	details
Proline + Oxoglutaric acid + Oxygen → 4-Hydroxyproline + Succinic acid + Carbon dioxide	details

References

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]

Enzyme Details

9. Gamma-butyrobetaine dioxygenase

General function:: Involved in iron ion binding
Specific function:: Catalyzes the formation of L-carnitine from gamma-butyrobetaine.
Gene Name:: BBOX1
Uniprot ID:: O75936
Molecular weight:: 44714.6

Reactions

4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen → L-Carnitine + Succinic acid + CO(2)	details
4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen → L-Carnitine + Succinic acid + Carbon dioxide	details

References

Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284 ]
Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423 ]
Yoshisue K, Yamamoto Y, Yoshida K, Saeki M, Minami Y, Esumi Y, Kawaguchi Y: Pharmacokinetics and biological fate of 3-(2,2, 2-trimethylhydrazinium)propionate dihydrate (MET-88), a novel cardioprotective agent, in rats. Drug Metab Dispos. 2000 Jun;28(6):687-94. [PubMed:10820142 ]

Enzyme Details

10. Procollagen-lysine,2-oxoglutarate 5-dioxygenase 1

General function:: Involved in oxidoreductase activity
Specific function:: Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens. These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links.
Gene Name:: PLOD1
Uniprot ID:: Q02809
Molecular weight:: 83549.55

Reactions

L-lysine-[procollagen] + Oxoglutaric acid + Oxygen → (2S,5R)-5-hydroxy-L-lysine-[procollagen] + Succinic acid + CO(2)	details
Protein lysine + Oxoglutaric acid + Oxygen → Procollagen 5-hydroxy-L-lysine + Succinic acid + Carbon dioxide + Water	details

References

Cudic M, Patel DA, Lauer-Fields JL, Brew K, Fields GB: Development of a convenient peptide-based assay for lysyl hydroxylase. Biopolymers. 2008;90(3):330-8. [PubMed:17610258 ]

Only showing the first 10 proteins. There are 44 proteins in total.

Show all enzymes and transporters

Showing metabocard for Succinic acid (HMDB0000254)

MOL for HMDB0000254 (Succinic acid)

3D MOL for HMDB0000254 (Succinic acid)

3D SDF for HMDB0000254 (Succinic acid)

3D-SDF for HMDB0000254 (Succinic acid)

PDB for HMDB0000254 (Succinic acid)

3D PDB for HMDB0000254 (Succinic acid)

SMILES for HMDB0000254 (Succinic acid)

INCHI for HMDB0000254 (Succinic acid)

Structure for HMDB0000254 (Succinic acid)

3D Structure for HMDB0000254 (Succinic acid)

Experimental Collision Cross Sections

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

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MS/MS Spectra

NMR Spectra

IR Spectra

Enzymes

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