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Showing metabocard for Succinic acid (HMDB0000254)

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enzymes (44) Show 44 proteins
Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2025-05-29 18:10:26 UTC
HMDB IDHMDB0000254
Secondary Accession Numbers
  • HMDB00254
Metabolite Identification
Common NameSuccinic acid
DescriptionSuccinic 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
Data?1676999679
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0000254 (Succinic acid)


  Mrv1652309032023582D          

  8  7  0  0  0  0            999 V2000
    2.0928    0.2063    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    5.6650   -0.2063    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.8072   -1.0311    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.9504    1.0311    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.5217    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.2361   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.8072   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.9504    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  7  1  0  0  0  0
  2  8  1  0  0  0  0
  3  7  2  0  0  0  0
  4  8  2  0  0  0  0
  5  6  1  0  0  0  0
  5  7  1  0  0  0  0
  6  8  1  0  0  0  0
M  END
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3D MOL for HMDB0000254 (Succinic acid)

HMDB0000254
     RDKit          3D
Succinic acid
 14 13  0  0  0  0  0  0  0  0999 V2000
    2.8686    0.7889   -0.7361 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.9035    0.2266   -0.1638 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.1747   -0.4955    0.9882 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.5430    0.3600   -0.7380 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3984   -0.4191    0.1635 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7892   -0.3809   -0.2775 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1591   -1.0952   -1.2456 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7536    0.4134    0.3186 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.6648   -0.2075    1.8241 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.4587   -0.0937   -1.7508 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.2383    1.4201   -0.8463 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0116   -1.4669    0.1728 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2699   -0.0395    1.1987 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4699    0.9892    1.0923 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  2  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  1  0
  6  7  2  0
  6  8  1  0
  3  9  1  0
  4 10  1  0
  4 11  1  0
  5 12  1  0
  5 13  1  0
  8 14  1  0
M  END
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3D SDF for HMDB0000254 (Succinic acid)


  Mrv1652309032023582D          

  8  7  0  0  0  0            999 V2000
    2.0928    0.2063    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    5.6650   -0.2063    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.8072   -1.0311    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.9504    1.0311    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.5217    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.2361   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.8072   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.9504    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  7  1  0  0  0  0
  2  8  1  0  0  0  0
  3  7  2  0  0  0  0
  4  8  2  0  0  0  0
  5  6  1  0  0  0  0
  5  7  1  0  0  0  0
  6  8  1  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000254

> <DATABASE_NAME>
hmdb

> <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

> <FORMULA>
C4H6O4

> <MOLECULAR_WEIGHT>
118.088

> <EXACT_MASS>
118.02660868

> <JCHEM_ACCEPTOR_COUNT>
4

> <JCHEM_ATOM_COUNT>
14

> <JCHEM_AVERAGE_POLARIZABILITY>
10.141334051490146

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
2

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
butanedioic acid

> <ALOGPS_LOGP>
-0.53

> <JCHEM_LOGP>
-0.3984829673333332

> <ALOGPS_LOGS>
0.25

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-2

> <JCHEM_PKA>
5.6869121792916

> <JCHEM_PKA_STRONGEST_ACIDIC>
3.5541665109763474

> <JCHEM_POLAR_SURFACE_AREA>
74.6

> <JCHEM_REFRACTIVITY>
23.537

> <JCHEM_ROTATABLE_BOND_COUNT>
3

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
2.11e+02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
succinic acid

> <JCHEM_VEBER_RULE>
0

$$$$
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3D-SDF for HMDB0000254 (Succinic acid)

HMDB0000254
     RDKit          3D
Succinic acid
 14 13  0  0  0  0  0  0  0  0999 V2000
    2.8686    0.7889   -0.7361 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.9035    0.2266   -0.1638 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.1747   -0.4955    0.9882 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.5430    0.3600   -0.7380 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3984   -0.4191    0.1635 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7892   -0.3809   -0.2775 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1591   -1.0952   -1.2456 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7536    0.4134    0.3186 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.6648   -0.2075    1.8241 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.4587   -0.0937   -1.7508 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.2383    1.4201   -0.8463 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0116   -1.4669    0.1728 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2699   -0.0395    1.1987 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4699    0.9892    1.0923 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  2  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  1  0
  6  7  2  0
  6  8  1  0
  3  9  1  0
  4 10  1  0
  4 11  1  0
  5 12  1  0
  5 13  1  0
  8 14  1  0
M  END
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PDB for HMDB0000254 (Succinic acid)

HEADER    PROTEIN                                 03-SEP-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   03-SEP-20         0                                  
HETATM    1  O   UNK     0       3.907   0.385   0.000  0.00  0.00           O+0
HETATM    2  O   UNK     0      10.575  -0.385   0.000  0.00  0.00           O+0
HETATM    3  O   UNK     0       5.240  -1.925   0.000  0.00  0.00           O+0
HETATM    4  O   UNK     0       9.241   1.925   0.000  0.00  0.00           O+0
HETATM    5  C   UNK     0       6.574   0.385   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0       7.907  -0.385   0.000  0.00  0.00           C+0
HETATM    7  C   UNK     0       5.240  -0.385   0.000  0.00  0.00           C+0
HETATM    8  C   UNK     0       9.241   0.385   0.000  0.00  0.00           C+0
CONECT    1    7                                                            
CONECT    2    8                                                            
CONECT    3    7                                                            
CONECT    4    8                                                            
CONECT    5    6    7                                                       
CONECT    6    5    8                                                       
CONECT    7    1    3    5                                                  
CONECT    8    2    4    6                                                  
MASTER        0    0    0    0    0    0    0    0    8    0   14    0
END
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3D PDB for HMDB0000254 (Succinic acid)

COMPND    HMDB0000254
HETATM    1  O1  UNL     1       2.869   0.789  -0.736  1.00  0.00           O  
HETATM    2  C1  UNL     1       1.903   0.227  -0.164  1.00  0.00           C  
HETATM    3  O2  UNL     1       2.175  -0.496   0.988  1.00  0.00           O  
HETATM    4  C2  UNL     1       0.543   0.360  -0.738  1.00  0.00           C  
HETATM    5  C3  UNL     1      -0.398  -0.419   0.163  1.00  0.00           C  
HETATM    6  C4  UNL     1      -1.789  -0.381  -0.277  1.00  0.00           C  
HETATM    7  O3  UNL     1      -2.159  -1.095  -1.246  1.00  0.00           O  
HETATM    8  O4  UNL     1      -2.754   0.413   0.319  1.00  0.00           O  
HETATM    9  H1  UNL     1       1.665  -0.207   1.824  1.00  0.00           H  
HETATM   10  H2  UNL     1       0.459  -0.094  -1.751  1.00  0.00           H  
HETATM   11  H3  UNL     1       0.238   1.420  -0.846  1.00  0.00           H  
HETATM   12  H4  UNL     1      -0.012  -1.467   0.173  1.00  0.00           H  
HETATM   13  H5  UNL     1      -0.270  -0.040   1.199  1.00  0.00           H  
HETATM   14  H6  UNL     1      -2.470   0.989   1.092  1.00  0.00           H  
CONECT    1    2    2
CONECT    2    3    4
CONECT    3    9
CONECT    4    5   10   11
CONECT    5    6   12   13
CONECT    6    7    7    8
CONECT    8   14
END
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SMILES for HMDB0000254 (Succinic acid)

OC(=O)CCC(O)=O
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INCHI for HMDB0000254 (Succinic acid)

InChI=1S/C4H6O4/c5-3(6)1-2-4(7)8/h1-2H2,(H,5,6)(H,7,8)
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View in JSmolView NMR AssignmentsView Stereo Labels
Synonyms
ValueSource
1,2-Ethanedicarboxylic acidChEBI
Acide butanedioiqueChEBI
Acide succiniqueChEBI
Acidum succinicumChEBI
Amber acidChEBI
AsuccinChEBI
BernsteinsaeureChEBI
ButandisaeureChEBI
Butanedionic acidChEBI
Dihydrofumaric acidChEBI
e363ChEBI
Ethylenesuccinic acidChEBI
HOOC-CH2-CH2-COOHChEBI
Spirit OF amberChEBI
1,2-EthanedicarboxylateGenerator
ButanedionateGenerator
DihydrofumarateGenerator
EthylenesuccinateGenerator
SuccinateGenerator
1,2 Ethanedicarboxylic acidMeSH
1,4 Butanedioic acidMeSH
1,4-Butanedioic acidMeSH
Ammonium succinateMeSH
Butanedioic acidMeSH
Potassium succinateMeSH
Succinate, ammoniumMeSH
Succinate, potassiumMeSH
1,4-ButanedioateHMDB
KatasuccinHMDB
Wormwood acidHMDB
2-Acetamido-2-deoxy-D-glucoseChEBI, HMDB
D-GlcNAcChEBI, HMDB
N-Acetyl-D-glucosamineChEBI, HMDB
N-AcetylchitosamineChEBI, HMDB
N Acetyl D glucosamineHMDB
2 Acetamido 2 deoxy D glucoseHMDB
2 Acetamido 2 deoxyglucoseHMDB
2-Acetamido-2-deoxyglucoseHMDB
AcetylglucosamineHMDB
Chemical FormulaC4H6O4
Average Molecular Weight118.088
Monoisotopic Molecular Weight118.02660868
IUPAC Namebutanedioic acid
Traditional Namesuccinic acid
CAS Registry Number110-15-6
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 KeyKDYFGRWQOYBRFD-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.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassDicarboxylic acids and derivatives
Direct ParentDicarboxylic 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 FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Health effectOrganoleptic effect
Disposition
Biological locationRoute of exposureSource
Process
Naturally occurring process
Role
Industrial applicationBiological role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point185 - 188 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility83.2 mg/mLNot Available
LogP-0.59HANSCH,C ET AL. (1995)
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M-H]-Baker119.84130932474
[M+H]+Baker122.65630932474
[M-H]-Not Available118.0http://allccs.zhulab.cn/database/detail?ID=AllCCS00001762
[M+H]+Not Available122.656http://allccs.zhulab.cn/database/detail?ID=AllCCS00001762
Predicted Molecular Properties
PropertyValueSource
Water Solubility211 g/LALOGPS
logP-0.53ALOGPS
logP-0.4ChemAxon
logS0.25ALOGPS
pKa (Strongest Acidic)3.55ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area74.6 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity23.54 m³·mol⁻¹ChemAxon
Polarizability10.14 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+124.47831661259
DarkChem[M-H]-117.77631661259
AllCCS[M+H]+128.06732859911
AllCCS[M-H]-123.25432859911
DeepCCS[M+H]+123.01330932474
DeepCCS[M-H]-120.23730932474
DeepCCS[M-2H]-156.60730932474
DeepCCS[M+Na]+131.53430932474
AllCCS[M+H]+128.132859911
AllCCS[M+H-H2O]+123.832859911
AllCCS[M+NH4]+132.032859911
AllCCS[M+Na]+133.132859911
AllCCS[M-H]-123.332859911
AllCCS[M+Na-2H]-126.432859911
AllCCS[M+HCOO]-129.932859911

Predicted Retention Times

Underivatized

Chromatographic MethodRetention TimeReference
Measured using a Waters Acquity ultraperformance liquid chromatography (UPLC) ethylene-bridged hybrid (BEH) C18 column (100 mm × 2.1 mm; 1.7 μmparticle diameter). Predicted by Afia on May 17, 2022. Predicted by Afia on May 17, 2022.1.38 minutes32390414
Predicted by Siyang on May 30, 20229.1546 minutes33406817
Predicted by Siyang using ReTip algorithm on June 8, 20224.52 minutes32390414
AjsUoB = Accucore 150 Amide HILIC with 10mM Ammonium Formate, 0.1% Formic Acid217.4 seconds40023050
Fem_Long = Waters ACQUITY UPLC HSS T3 C18 with Water:MeOH and 0.1% Formic Acid849.5 seconds40023050
Fem_Lipids = Ascentis Express C18 with (60:40 water:ACN):(90:10 IPA:ACN) and 10mM NH4COOH + 0.1% Formic Acid339.8 seconds40023050
Life_Old = Waters ACQUITY UPLC BEH C18 with Water:(20:80 acetone:ACN) and 0.1% Formic Acid64.4 seconds40023050
Life_New = RP Waters ACQUITY UPLC HSS T3 C18 with Water:(30:70 MeOH:ACN) and 0.1% Formic Acid219.7 seconds40023050
RIKEN = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid68.5 seconds40023050
Eawag_XBridgeC18 = XBridge C18 3.5u 2.1x50 mm with Water:MeOH and 0.1% Formic Acid261.3 seconds40023050
BfG_NTS_RP1 =Agilent Zorbax Eclipse Plus C18 (2.1 mm x 150 mm, 3.5 um) with Water:ACN and 0.1% Formic Acid274.4 seconds40023050
HILIC_BDD_2 = Merck SeQuant ZIC-HILIC with ACN(0.1% formic acid):water(16 mM ammonium formate)294.8 seconds40023050
UniToyama_Atlantis = RP Waters Atlantis T3 (2.1 x 150 mm, 5 um) with ACN:Water and 0.1% Formic Acid595.0 seconds40023050
BDD_C18 = Hypersil Gold 1.9µm C18 with Water:ACN and 0.1% Formic Acid107.9 seconds40023050
UFZ_Phenomenex = Kinetex Core-Shell C18 2.6 um, 3.0 x 100 mm, Phenomenex with Water:MeOH and 0.1% Formic Acid799.1 seconds40023050
SNU_RIKEN_POS = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid222.7 seconds40023050
RPMMFDA = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid246.1 seconds40023050
MTBLS87 = Merck SeQuant ZIC-pHILIC column with ACN:Water and :ammonium carbonate787.2 seconds40023050
KI_GIAR_zic_HILIC_pH2_7 = Merck SeQuant ZIC-HILIC with ACN:Water and 0.1% FA268.2 seconds40023050
Meister zic-pHILIC pH9.3 = Merck SeQuant ZIC-pHILIC column with ACN:Water 5mM NH4Ac pH9.3 and 5mM ammonium acetate in water460.4 seconds40023050

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
Succinic acidOC(=O)CCC(O)=O1925.6Standard polar33892256
Succinic acidOC(=O)CCC(O)=O853.5Standard non polar33892256
Succinic acidOC(=O)CCC(O)=O1214.2Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Succinic acid,1TMS,isomer #1C[Si](C)(C)OC(=O)CCC(=O)O1238.9Semi standard non polar33892256
Succinic acid,2TMS,isomer #1C[Si](C)(C)OC(=O)CCC(=O)O[Si](C)(C)C1321.3Semi standard non polar33892256
Succinic acid,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CCC(=O)O1497.1Semi standard non polar33892256
Succinic acid,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CCC(=O)O[Si](C)(C)C(C)(C)C1743.0Semi standard non polar33892256
Spectra
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
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified16.0 (0.00-32.0) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified8.8 +/- 2.7 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
 details
BloodDetected and Quantified23.5 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified6.10-11.5 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
 details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
BloodDetected and Quantified1.59 ± 0.69 uMAdult (>18 years old)Both
Normal
    • Quantitative comp...
 details
BloodDetected and Quantified1.4 ± 0.88 uMAdult (>18 years old)Both
Normal
    • Quantitative comp...
 details
BloodDetected and Quantified1.1 ± 0.19 uMAdult (>18 years old)Both
Normal
    • Quantitative comp...
 details
BloodDetected and Quantified1.43 ± 0.33 uMAdult (>18 years old)Both
Normal
    • Quantitative comp...
 details
BloodDetected and Quantified1.76 ± 0.29 uMAdult (>18 years old)Both
Normal
    • Quantitative comp...
 details
BloodDetected and Quantified1.06 ± 0.48 uMAdult (>18 years old)Both
Normal
    • Quantitative comp...
 details
BloodDetected and Quantified2.98 ± 1 uMAdult (>18 years old)Both
Normal
    • Quantitative comp...
 details
BloodDetected and Quantified2.03 ± 0.43 uMAdult (>18 years old)Both
Normal
    • Quantitative comp...
 details
BloodDetected and Quantified1.54 ± 0.7 uMAdult (>18 years old)Both
Normal
    • Quantitative comp...
 details
Breast MilkDetected and Quantified8.4 +/- 2.9 uMAdult (>18 years old)Female
Normal		 details
Cerebrospinal Fluid (CSF)Detected and Quantified28.5 (24-33) uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
 details
Cerebrospinal Fluid (CSF)Detected and Quantified19 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified150.0 +/- 110.0 uMElderly (>65 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified47.2 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified3.0 +/- 2.0 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified3 +/- 2 uMAdult (>18 years old)BothNormal details
FecesDetected and Quantified132.528 +/- 151.836 nmol/g wet fecesNot SpecifiedNot Specified
Normal		 details
FecesDetected and Quantified1633.528 +/- 1822.370 nmol/g wet fecesNot SpecifiedNot Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not Specified
Normal		 details
FecesDetected and Quantified255 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal		 details
FecesDetected and Quantified222 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal		 details
FecesDetected and Quantified250 nmol/g wet fecesAdult (>18 years old)Not Specified
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
SalivaDetected and Quantified2260 (60.0-4460) uMAdult (>18 years old)BothNormal details
SalivaDetected and Quantified4-145 uMAdult (>18 years old)Male
normal		 details
SalivaDetected and Quantified1-33 uMAdult (>18 years old)Male
normal		 details
SalivaDetected and Quantified125.15 +/- 180.60 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
SalivaDetected and Quantified14 (2-112) uMAdult (>18 years old)Male
Normal		 details
SalivaDetected and Quantified9 (1-78) uMAdult (>18 years old)Male
Normal		 details
SalivaDetected and Quantified21 (<1-66) uMAdult (>18 years old)Male
Normal		 details
SalivaDetected and Quantified9 (<1-54) uMAdult (>18 years old)Female
Normal		 details
SalivaDetected and Quantified15.0 +/- 15.5 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
 details
SalivaDetected and Quantified15.6 +/- 27.7 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
 details
SalivaDetected and Quantified24.3 +/- 20.4 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
 details
SalivaDetected and Quantified9.03 +/- 5.22 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
 details
SweatDetected but not QuantifiedNot QuantifiedAdult BothNormal details
UrineDetected and Quantified7.7 (1.9-20.0) umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified197.2 (29.4-486.2) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified185.4 (6.0-342.6) umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified11.6 (4.0-27.3) umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified8.25 (0.5-16.0) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified9.9 +/- 5.0 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified14.4 +/- 4.9 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified12.60 +/- 12.13 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified3.8 (1.25-6.7) umol/mmol creatinineAdult (>18 years old)BothNormal
    • Geigy Scientific ...
 details
UrineDetected and Quantified2.10 umol/mmol creatinineAdult (>18 years old)BothNormal
    • Shaykhutdinov RA,...
 details
UrineDetected and Quantified14.48 +/- 3.20 umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified7.5 (0.5-16) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified5.6 +/- 3.8 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified1.3 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified6.2 (2.5-13.5) umol/mmol creatinineAdult (>18 years old)Both
Normal		 details
UrineDetected and Quantified4.7 (1.1-14.5) umol/mmol creatinineAdult (>18 years old)Both
Normal		 details
UrineDetected and Quantified6.0 (0.3-33.3) umol/mmol creatinineAdult (>18 years old)Both
Normal		 details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal		 details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified14.581 +/- 9.663 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
 details
UrineDetected and Quantified163.491 +/- 191.759 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
 details
UrineDetected and Quantified<87.14 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
 details
UrineDetected and Quantified1.4-2.3 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified1.4-1.7 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified<335.0976 umol/mmol creatinineInfant (0-1 year old)MaleNormal details
UrineDetected and Quantified1.915-71.807 umol/mmol creatinineChildren (1 - 13 years old)MaleNormal details
UrineDetected and Quantified<76 umol/mmol creatinineNot SpecifiedNot Specified
Normal		 details
UrineDetected and Quantified<97 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified110 umol/mmol creatinineNewborn (0-30 days old)Both
Normal		 details
UrineDetected and Quantified9-360 umol/mmol creatinineNewborn (0-30 days old)Both
Normal		 details
UrineDetected and Quantified9.4 +/- 9.2 umol/mmol creatinineNewborn (0-30 days old)Both
Normal		 details
UrineDetected and Quantified11.9-24.1 umol/mmol creatinineInfant (0-1 year old)Female
Normal		 details
UrineDetected and Quantified15.7-69.5 umol/mmol creatinineInfant (0-1 year old)Male
Normal		 details
UrineDetected and Quantified20.2-49.6 umol/mmol creatinineInfant (0-1 year old)Female
Normal		 details
UrineDetected and Quantified22.0-42.0 umol/mmol creatinineInfant (0-1 year old)Male
Normal		 details
UrineDetected and Quantified4.35-81.2 umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified16.99(5.66-67.50) umol/mmol creatinineNewborn (0-30 days old)FemaleNormal details
UrineDetected and Quantified16.76(3.97-85.17) umol/mmol creatinineNewborn (0-30 days old)MaleNormal details
UrineDetected and Quantified111.02 (31.39-241.29) umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • Analysis of 40 NI...
 details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified3.31 (1.13) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified3.35 (1.78) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect details
BloodDetected and Quantified6.50 +/- 10.83 uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified6.90 +/- 6.33 uMAdult (>18 years old)Female
Pregnancy with fetuses with trisomy 18		 details
BloodDetected and Quantified5.6 (12.5) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified5.3 (5.6) uMAdult (>18 years old)FemaleDown syndrome pregnancy details
BloodDetected and Quantified12.4 (13.9) uMAdult (>18 years old)FemalePregnancy details
BloodDetected and Quantified4.3 (1.6) uMAdult (>18 years old)FemaleEarly preeclampsia details
Cerebrospinal Fluid (CSF)Detected and Quantified55.0 (19.0-91.0) uMAdult (>18 years old)BothAlzheimer's disease details
Cerebrospinal Fluid (CSF)Detected and Quantified19.0 (0.0-38.0) uMAdult (>18 years old)BothCanavan Disease details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothCrohn's disease details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Not SpecifiedGenetically At-Risk of celiac disease details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedCrohns disease details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedUlcerative colitis details
FecesDetected but not QuantifiedNot QuantifiedChildren (6 - 18 years old)Not SpecifiedUnclassified IBD details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleGout details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleMyalgic encephalomyelitis/chronic fatigue syndrome details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Irritable bowel syndrome		 details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
Cryptosporidium infection		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Ulcerative colitis		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MalePeriodontal Probing Depth details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleAttachment loss  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleMissing teeth details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Bladder cancer		 details
UrineDetected and Quantified43.35 +/- 25.08 umol/mmol creatinineChildren (1-13 years old)BothMalnutrition (type kwashiorkor and marasmus) details
UrineDetected and Quantified15 umol/mmol creatinineChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency details
UrineDetected and Quantified75-185 umol/mmol creatinineInfant (0-1 year old)Male
2-Ketoglutarate dehydrogenase complex deficiency		 details
UrineDetected and Quantified749-784 umol/mmol creatinineInfant (0-1 year old)MaleD-2-hydroxyglutaric aciduria details
UrineDetected and Quantified141.698 umol/mmol creatinineInfant (0-1 year old)MaleFumarase deficiency details
UrineDetected and Quantified86.168 umol/mmol creatinineChildren (1 - 13 years old)MaleD-2-Hydroxyglutaric aciduria details
UrineDetected and Quantified50 umol/mmol creatinineInfant (0-1 year old)MaleMalonyl-Coa decarboxylase deficiency details
UrineDetected and Quantified6.667 umol/mmol creatinineChildren (1 - 13 years old)MaleD-2-Hydroxyglutaric aciduria details
UrineDetected and Quantified4.787 umol/mmol creatinineChildren (1 - 13 years old)MaleD-2-Hydroxyglutaric aciduria details
UrineDetected and Quantified9.0 +/- 6.0 umol/mmol creatinineAdult (>18 years old)BothLung cancer details
UrineDetected and Quantified530.857 +/- 876.714 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Gastroesophageal reflux disease
    • Analysis of 30 no...
 details
UrineDetected and Quantified27.897 +/- 29.998 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
 details
UrineDetected and Quantified467.612 +/- 705.456 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
 details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)FemaleEpithelial ovarian cancer details
Associated Disorders and Diseases
Disease References
Pregnancy
  1. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
Early preeclampsia
  1. 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 ]
Alzheimer's disease
  1. 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 ]
Canavan disease
  1. 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 ]
Irritable bowel syndrome
  1. 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
  1. 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 ]
  2. 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
  1. 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 ]
  2. 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
  1. 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
  1. 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
  1. 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
  1. Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Colorectal cancer
  1. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 ]
Lung Cancer
  1. 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 ]
D-2-hydroxyglutaric aciduria
  1. 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 ]
  2. 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
  1. 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
  1. 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
  1. 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
  1. 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
DrugBank IDDB00139
Phenol Explorer Compound IDNot Available
FooDB IDFDB001931
KNApSAcK IDC00001205
Chemspider ID1078
KEGG Compound IDC00042
BioCyc IDSUC
BiGG IDNot Available
Wikipedia LinkSuccinic_acid
METLIN IDNot Available
PubChem Compound1738118
PDB IDNot Available
ChEBI ID15741
Food Biomarker OntologyNot Available
VMH IDSUCC
MarkerDB IDMDB00000124
Good Scents IDNot Available
References
Synthesis ReferenceBerglund, Kris Arvid; Andersson, Christian; Rova, Ulrika. Process for the production of succinic acid. PCT Int. Appl. (2007), 30pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 ]
  6. 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 ]
  7. 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 ]
  8. 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 ]
  9. 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 ]
  10. 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 ]
  11. 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 ]
  12. 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 ]
  13. 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 ]
  14. 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 ]
  15. 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 ]
  16. 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 ]
  17. 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 ]
  18. 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 ]
  19. 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 ]
  20. 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 ]
  21. 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 aciddetails
References
  1. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 aciddetails
References
  1. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 aciddetails
References
  1. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 aciddetails
References
  1. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 + NADHdetails
Succinic acid semialdehyde + NAD + Water → Succinic acid + NADH + Hydrogen Iondetails
References
  1. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 ]
  6. 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 dioxidedetails
References
  1. 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 ]
  2. 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 dioxidedetails
References
  1. 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 ]
  2. 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 dioxidedetails
References
  1. 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 ]
  2. 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 ]
  3. 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 + Waterdetails
References
  1. 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