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Showing metabocard for 2-Hydroxyglutarate (HMDB0059655)

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enzymes (4) Show 4 proteins
Record Information
Version5.0
StatusDetected and Quantified
Creation Date2012-10-30 10:32:48 UTC
Update Date2023-05-30 20:55:56 UTC
HMDB IDHMDB0059655
Secondary Accession Numbers
  • HMDB59655
Metabolite Identification
Common Name2-Hydroxyglutarate
Description2-Hydroxyglutarate exists in 2 isomers: L-2-hydroxyglutarate acid and D-2-hydroxyglutarate. Both the D and the L stereoisomers of hydroxyglutaric acid (EC 1.1.99.2) are found in body fluids. In humans it is part of butanoate metabolic pathway and can be produced by phosphoglycerate dehydrogenase (PHGDH). More specifically, the enzyme PHGDH catalyzes the NADH-dependent reduction of ?-ketoglutarate (AKG) to D-2-hydroxyglutarate (D-2HG). 2-hydroxyglutarate is also the product of gain-of-function mutations in the cytosolic and mitochondrial isoforms of isocitrate dehydrogenase (IDH). Additionally, 2-hydroxyglutarate can be converted to ?-ketoglutaric acid through the action of 2-hydroxyglutarate dehydrogenase (HGDH). Humans have to variants of this enzyme: D-2-hydroxyglutarate dehydrogenase (D2HGDH) and L-2-hydroxyglutarate dehydrogenase (L2HGDH). A deficiency in either of these two enzymes can lead to a disease known as 2-hydroxyglutaric aciduria. L-2-hydroxyglutaric aciduria (caused by loss of L2HGDH) is chronic, with early symptoms such as hypotonia, tremors, and epilepsy declining into spongiform leukoencephalopathy, muscular choreodystonia, mental retardation, and psychomotor regression. D-2-hydroxyglutaric aciduria (caused by loss of D2HGDH or gain of function of IDH) is rare, with symptoms including cancer, macrocephaly, cardiomyopathy, mental retardation, hypotonia, and cortical blindness. 2-hydroxyglutarate was the first oncometabolite (or cancer-causing metabolite) to be formally named or identified. In cancer it is either produced by overexpression of phosphoglycerate dehydrogenase (PHGDH) or is produced in excess by gain-of-function mutations in the cytosolic and mitochondrial isoforms of isocitrate dehydrogenase (IDH). IDH is part of TCA cycle and is generated in high abundance when IDH is mutated. 2-hydroxyglutarate is sufficiently similar in structure to 2-oxogluratate (2OG) that it is able to inhibit a range of 2OG-dependent dioxygenases, including histone lysine demethylases (KDMs) and members of the ten-eleven translocation (TET) family of 5-methylcytosine (5mC) hydroxylases. This inhibitory effect leads to alterations in the hypoxia induced factor (HIF)-mediated hypoxic response and alterations in gene expression through global epigenetic remodeling. The net effect is that 2-hydroxyglutarate causes a cascading effect that leads genetic perturbations and malignant transformation. Furthermore, 2-hydroxyglutarate is found to be associated with glutaric aciduria II, which is also an inborn error of metabolism. 2-Hydroxyglutarate has also been found to be a metabolite in Aspergillus (PMID: 6057807 ).
Structure
Data?1563865961
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0059655 (2-Hydroxyglutarate)


  Mrv1652309042000402D          

 10  9  0  0  0  0            999 V2000
 9999.2646 9999.7892    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9999.979310000.2013    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10000.6939 9999.7892    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10001.411510000.2013    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10002.1250 9999.7892    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10001.411510001.0261    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.549810000.2013    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9997.8349 9999.7892    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.549810001.0261    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9999.2646 9998.9638    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  7  1  0  0  0  0
  1 10  1  0  0  0  0
  2  3  1  0  0  0  0
  3  4  1  0  0  0  0
  4  5  1  0  0  0  0
  4  6  2  0  0  0  0
  7  8  1  0  0  0  0
  7  9  2  0  0  0  0
M  END
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3D MOL for HMDB0059655 (2-Hydroxyglutarate)

HMDB0059655
     RDKit          3D
2-Hydroxyglutarate
 18 17  0  0  0  0  0  0  0  0999 V2000
    3.0317   -1.0607    0.2792 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.3638   -0.0196    0.1451 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.8559    1.1002   -0.5141 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.9655    0.0897    0.6959 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.1013   -0.6685   -0.2877 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3473   -0.6983    0.0686 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9678   -1.4456   -0.9647 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9167    0.6701    0.0149 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7653    0.9961   -0.8852 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5910    1.6824    0.9064 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.5718    1.0602   -1.2099 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.9122   -0.3792    1.7028 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.6495    1.1467    0.7231 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.1879   -0.0894   -1.2531 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.5364   -1.6704   -0.4715 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5183   -1.1992    1.0381 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5178   -2.1661   -0.5702 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5517    2.6517    0.5823 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  1  0
  6  8  1  0
  8  9  2  0
  8 10  1  0
  3 11  1  0
  4 12  1  0
  4 13  1  0
  5 14  1  0
  5 15  1  0
  6 16  1  0
  7 17  1  0
 10 18  1  0
M  END
Download

3D SDF for HMDB0059655 (2-Hydroxyglutarate)


  Mrv1652309042000402D          

 10  9  0  0  0  0            999 V2000
 9999.2646 9999.7892    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9999.979310000.2013    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10000.6939 9999.7892    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10001.411510000.2013    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10002.1250 9999.7892    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10001.411510001.0261    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.549810000.2013    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9997.8349 9999.7892    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.549810001.0261    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9999.2646 9998.9638    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  7  1  0  0  0  0
  1 10  1  0  0  0  0
  2  3  1  0  0  0  0
  3  4  1  0  0  0  0
  4  5  1  0  0  0  0
  4  6  2  0  0  0  0
  7  8  1  0  0  0  0
  7  9  2  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0059655

> <DATABASE_NAME>
hmdb

> <SMILES>
OC(CCC(O)=O)C(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C5H8O5/c6-3(5(9)10)1-2-4(7)8/h3,6H,1-2H2,(H,7,8)(H,9,10)

> <INCHI_KEY>
HWXBTNAVRSUOJR-UHFFFAOYSA-N

> <FORMULA>
C5H8O5

> <MOLECULAR_WEIGHT>
148.114

> <EXACT_MASS>
148.037173366

> <JCHEM_ACCEPTOR_COUNT>
5

> <JCHEM_ATOM_COUNT>
18

> <JCHEM_AVERAGE_POLARIZABILITY>
12.97429694878232

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
3

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
2-hydroxypentanedioic acid

> <ALOGPS_LOGP>
-1.04

> <JCHEM_LOGP>
-0.824980223

> <ALOGPS_LOGS>
0.03

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-2

> <JCHEM_PKA>
4.159324461588801

> <JCHEM_PKA_STRONGEST_ACIDIC>
3.276485914460605

> <JCHEM_PKA_STRONGEST_BASIC>
-3.8068384384876675

> <JCHEM_POLAR_SURFACE_AREA>
94.83000000000001

> <JCHEM_REFRACTIVITY>
29.6302

> <JCHEM_ROTATABLE_BOND_COUNT>
4

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
1.57e+02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
2-hydroxyglutaric acid

> <JCHEM_VEBER_RULE>
0

$$$$
Download

3D-SDF for HMDB0059655 (2-Hydroxyglutarate)

HMDB0059655
     RDKit          3D
2-Hydroxyglutarate
 18 17  0  0  0  0  0  0  0  0999 V2000
    3.0317   -1.0607    0.2792 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.3638   -0.0196    0.1451 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.8559    1.1002   -0.5141 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.9655    0.0897    0.6959 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.1013   -0.6685   -0.2877 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3473   -0.6983    0.0686 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9678   -1.4456   -0.9647 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9167    0.6701    0.0149 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7653    0.9961   -0.8852 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5910    1.6824    0.9064 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.5718    1.0602   -1.2099 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.9122   -0.3792    1.7028 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.6495    1.1467    0.7231 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.1879   -0.0894   -1.2531 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.5364   -1.6704   -0.4715 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5183   -1.1992    1.0381 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5178   -2.1661   -0.5702 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5517    2.6517    0.5823 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  1  0
  6  8  1  0
  8  9  2  0
  8 10  1  0
  3 11  1  0
  4 12  1  0
  4 13  1  0
  5 14  1  0
  5 15  1  0
  6 16  1  0
  7 17  1  0
 10 18  1  0
M  END
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PDB for HMDB0059655 (2-Hydroxyglutarate)

HEADER    PROTEIN                                 04-SEP-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   04-SEP-20         0                                  
HETATM    1  C   UNK     0    8665.2948666.273   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0    8666.6288667.042   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0    8667.9628666.273   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0    8669.3018667.042   0.000  0.00  0.00           C+0
HETATM    5  O   UNK     0    8670.6338666.273   0.000  0.00  0.00           O+0
HETATM    6  O   UNK     0    8669.3018668.582   0.000  0.00  0.00           O+0
HETATM    7  C   UNK     0    8663.9608667.042   0.000  0.00  0.00           C+0
HETATM    8  O   UNK     0    8662.6258666.273   0.000  0.00  0.00           O+0
HETATM    9  O   UNK     0    8663.9608668.582   0.000  0.00  0.00           O+0
HETATM   10  O   UNK     0    8665.2948664.732   0.000  0.00  0.00           O+0
CONECT    1    2    7   10                                                  
CONECT    2    1    3                                                       
CONECT    3    2    4                                                       
CONECT    4    3    5    6                                                  
CONECT    5    4                                                            
CONECT    6    4                                                            
CONECT    7    1    8    9                                                  
CONECT    8    7                                                            
CONECT    9    7                                                            
CONECT   10    1                                                            
MASTER        0    0    0    0    0    0    0    0   10    0   18    0
END
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3D PDB for HMDB0059655 (2-Hydroxyglutarate)

COMPND    HMDB0059655
HETATM    1  O1  UNL     1       3.032  -1.061   0.279  1.00  0.00           O  
HETATM    2  C1  UNL     1       2.364  -0.020   0.145  1.00  0.00           C  
HETATM    3  O2  UNL     1       2.856   1.100  -0.514  1.00  0.00           O  
HETATM    4  C2  UNL     1       0.966   0.090   0.696  1.00  0.00           C  
HETATM    5  C3  UNL     1       0.101  -0.669  -0.288  1.00  0.00           C  
HETATM    6  C4  UNL     1      -1.347  -0.698   0.069  1.00  0.00           C  
HETATM    7  O3  UNL     1      -1.968  -1.446  -0.965  1.00  0.00           O  
HETATM    8  C5  UNL     1      -1.917   0.670   0.015  1.00  0.00           C  
HETATM    9  O4  UNL     1      -2.765   0.996  -0.885  1.00  0.00           O  
HETATM   10  O5  UNL     1      -1.591   1.682   0.906  1.00  0.00           O  
HETATM   11  H1  UNL     1       3.572   1.060  -1.210  1.00  0.00           H  
HETATM   12  H2  UNL     1       0.912  -0.379   1.703  1.00  0.00           H  
HETATM   13  H3  UNL     1       0.650   1.147   0.723  1.00  0.00           H  
HETATM   14  H4  UNL     1       0.188  -0.089  -1.253  1.00  0.00           H  
HETATM   15  H5  UNL     1       0.536  -1.670  -0.472  1.00  0.00           H  
HETATM   16  H6  UNL     1      -1.518  -1.199   1.038  1.00  0.00           H  
HETATM   17  H7  UNL     1      -2.518  -2.166  -0.570  1.00  0.00           H  
HETATM   18  H8  UNL     1      -1.552   2.652   0.582  1.00  0.00           H  
CONECT    1    2    2
CONECT    2    3    4
CONECT    3   11
CONECT    4    5   12   13
CONECT    5    6   14   15
CONECT    6    7    8   16
CONECT    7   17
CONECT    8    9    9   10
CONECT   10   18
END
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SMILES for HMDB0059655 (2-Hydroxyglutarate)

OC(CCC(O)=O)C(O)=O
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INCHI for HMDB0059655 (2-Hydroxyglutarate)

InChI=1S/C5H8O5/c6-3(5(9)10)1-2-4(7)8/h3,6H,1-2H2,(H,7,8)(H,9,10)
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View in JSmolView NMR AssignmentsView Stereo Labels
Synonyms
ValueSource
alpha-Hydroxyglutaric acidChEBI
alpha-HydroxyglutarateKegg
a-HydroxyglutarateGenerator
a-Hydroxyglutaric acidGenerator
Α-hydroxyglutarateGenerator
Α-hydroxyglutaric acidGenerator
2-Hydroxyglutaric acidGenerator
D-2-HydroxyglutarateHMDB
alpha-Hydroxyglutarate, (D)-isomerHMDB
alpha-Hydroxyglutarate, (DL)-isomerHMDB
alpha-Hydroxyglutarate, (L)-isomerHMDB
alpha-Hydroxyglutarate, disodium saltHMDB
(±)-2-hydroxyglutaric acidHMDB
2,3-Dideoxypentaric acidHMDB
2-Hydroxypentanedioic acidHMDB
DL-2-Hydroxyglutaric acidHMDB
2-HydroxyglutarateGenerator
Chemical FormulaC5H8O5
Average Molecular Weight148.114
Monoisotopic Molecular Weight148.037173366
IUPAC Name2-hydroxypentanedioic acid
Traditional Name2-hydroxyglutaric acid
CAS Registry Number2889-31-8
SMILES
OC(CCC(O)=O)C(O)=O
InChI Identifier
InChI=1S/C5H8O5/c6-3(5(9)10)1-2-4(7)8/h3,6H,1-2H2,(H,7,8)(H,9,10)
InChI KeyHWXBTNAVRSUOJR-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as short-chain hydroxy acids and derivatives. These are hydroxy acids with an alkyl chain the contains less than 6 carbon atoms.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassHydroxy acids and derivatives
Sub ClassShort-chain hydroxy acids and derivatives
Direct ParentShort-chain hydroxy acids and derivatives
Alternative Parents
Substituents
  • Short-chain hydroxy acid
  • Fatty acid
  • Monosaccharide
  • Dicarboxylic acid or derivatives
  • Alpha-hydroxy acid
  • Secondary alcohol
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Alcohol
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effectNot Available
Disposition
Biological locationSource
Process
Naturally occurring process
Role
Industrial application
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility157 g/LALOGPS
logP-1ALOGPS
logP-0.82ChemAxon
logS0.03ALOGPS
pKa (Strongest Acidic)3.28ChemAxon
pKa (Strongest Basic)-3.8ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area94.83 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity29.63 m³·mol⁻¹ChemAxon
Polarizability12.97 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]+132.05631661259
DarkChem[M-H]-125.70531661259
DeepCCS[M+H]+124.88830932474
DeepCCS[M-H]-121.81530932474
DeepCCS[M-2H]-158.68630932474
DeepCCS[M+Na]+133.5430932474
AllCCS[M+H]+134.032859911
AllCCS[M+H-H2O]+130.032859911
AllCCS[M+NH4]+137.832859911
AllCCS[M+Na]+138.932859911
AllCCS[M-H]-126.532859911
AllCCS[M+Na-2H]-128.732859911
AllCCS[M+HCOO]-131.132859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
2-HydroxyglutarateOC(CCC(O)=O)C(O)=O2380.1Standard polar33892256
2-HydroxyglutarateOC(CCC(O)=O)C(O)=O1079.9Standard non polar33892256
2-HydroxyglutarateOC(CCC(O)=O)C(O)=O1440.1Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
2-Hydroxyglutarate,1TMS,isomer #1C[Si](C)(C)OC(CCC(=O)O)C(=O)O1460.3Semi standard non polar33892256
2-Hydroxyglutarate,1TMS,isomer #2C[Si](C)(C)OC(=O)CCC(O)C(=O)O1446.5Semi standard non polar33892256
2-Hydroxyglutarate,1TMS,isomer #3C[Si](C)(C)OC(=O)C(O)CCC(=O)O1433.0Semi standard non polar33892256
2-Hydroxyglutarate,2TMS,isomer #1C[Si](C)(C)OC(=O)CCC(O[Si](C)(C)C)C(=O)O1553.8Semi standard non polar33892256
2-Hydroxyglutarate,2TMS,isomer #2C[Si](C)(C)OC(=O)C(CCC(=O)O)O[Si](C)(C)C1522.6Semi standard non polar33892256
2-Hydroxyglutarate,2TMS,isomer #3C[Si](C)(C)OC(=O)CCC(O)C(=O)O[Si](C)(C)C1484.7Semi standard non polar33892256
2-Hydroxyglutarate,3TMS,isomer #1C[Si](C)(C)OC(=O)CCC(O[Si](C)(C)C)C(=O)O[Si](C)(C)C1587.9Semi standard non polar33892256
2-Hydroxyglutarate,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(CCC(=O)O)C(=O)O1707.9Semi standard non polar33892256
2-Hydroxyglutarate,1TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)CCC(O)C(=O)O1691.3Semi standard non polar33892256
2-Hydroxyglutarate,1TBDMS,isomer #3CC(C)(C)[Si](C)(C)OC(=O)C(O)CCC(=O)O1695.5Semi standard non polar33892256
2-Hydroxyglutarate,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CCC(O[Si](C)(C)C(C)(C)C)C(=O)O1996.7Semi standard non polar33892256
2-Hydroxyglutarate,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)C(CCC(=O)O)O[Si](C)(C)C(C)(C)C1974.2Semi standard non polar33892256
2-Hydroxyglutarate,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)OC(=O)CCC(O)C(=O)O[Si](C)(C)C(C)(C)C1940.9Semi standard non polar33892256
2-Hydroxyglutarate,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CCC(O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2206.6Semi standard non polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxyglutarate GC-MS (Non-derivatized) - 70eV, Positivesplash10-0uel-9300000000-fbe125fbbc3b5bc0783b2016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxyglutarate GC-MS (3 TMS) - 70eV, Positivesplash10-00gv-8191000000-f6fdffd87ffe1acf65b42017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Hydroxyglutarate GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 0V, negative-QTOFsplash10-0002-0900000000-09163ea46c418972472c2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 1V, negative-QTOFsplash10-0002-0900000000-d04b995445fdf4b9a53d2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 1V, negative-QTOFsplash10-0002-0900000000-b0a25d5d220611188e252020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 1V, negative-QTOFsplash10-0002-0900000000-fffbc099f0f67f0511f32020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 2V, negative-QTOFsplash10-0002-0900000000-09f10084fba5e519de0a2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 2V, negative-QTOFsplash10-002b-0900000000-dff435b3dcd9563204792020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 3V, negative-QTOFsplash10-004j-0900000000-bda2b170e24c7a9e5c352020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 3V, negative-QTOFsplash10-004i-0900000000-73ae87a8fb9bc7ee539d2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 4V, negative-QTOFsplash10-0fb9-1900000000-b1dc0a848bf8324490a72020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 5V, negative-QTOFsplash10-0ug0-3900000000-07793b211b4728e176692020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 6V, negative-QTOFsplash10-0udr-6900000000-f034f6e5155f6da3db5a2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate n/a 10V, negative-QTOFsplash10-004i-0900000000-80c3e765d7538561ccbf2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate n/a 10V, negative-QTOFsplash10-000i-9300000000-7ffbc20d629772e358cc2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 1V, negative-QTOFsplash10-004i-0900000000-982cc580a7d58e352fcb2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 1V, negative-QTOFsplash10-004i-1900000000-5920ffef63672e32cc732020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 2V, negative-QTOFsplash10-004i-2900000000-0945bfdbb47d1208ca552020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 2V, negative-QTOFsplash10-004r-3900000000-8a092cb937763eb031bb2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 3V, negative-QTOFsplash10-004r-6900000000-41fa2c2ee20825e5b11d2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 4V, negative-QTOFsplash10-002r-9800000000-6677f366953c5f35a17b2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 4V, negative-QTOFsplash10-000i-9500000000-0ca597332561395d2cc32020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 5V, negative-QTOFsplash10-000i-9400000000-510a9566cf27424fa58c2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate n/a 10V, negative-QTOFsplash10-0udr-4900000000-cf48fcc4c8561ba5a0e02020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate LC-ESI-QTOF 35V, positive-QTOFsplash10-00di-9000000000-36768b96f759cfee016d2020-07-21HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate n/a 10V, positive-QTOFsplash10-0udi-0900000000-47e5cb48d7b683b187472020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Hydroxyglutarate Orbitrap 1V, positive-QTOFsplash10-03dj-1790000000-8eb0579a5917747d340e2020-07-22HMDB team, MONAView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-24Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Brain
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.7 +/- 0.2 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified1.5 (0.0-1.5) uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified1 uMChildren (1-13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified1.0 +/- 1/0 uMAdult (>18 years old)Not SpecifiedNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Not SpecifiedNormal 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)Both
Normal		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
SalivaDetected and Quantified0.881 +/- 1.13 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
 details
SalivaDetected and Quantified1.08 +/- 0.629 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
 details
SalivaDetected and Quantified1.44 +/- 1.33 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
 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)Both
Normal
    • Zerihun T. Dame, ...
 details
UrineDetected and Quantified<5 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified0-1.2 umol/mmol creatinineInfant (0-1 year old)Female
Normal		 details
UrineDetected and Quantified1.0-3.7 umol/mmol creatinineInfant (0-1 year old)Male
Normal		 details
UrineDetected and Quantified1.0-4 umol/mmol creatinineInfant (0-1 year old)Male
Normal		 details
UrineDetected and Quantified1.2-5.1 umol/mmol creatinineInfant (0-1 year old)Female
Normal		 details
UrineDetected and Quantified18.25+/- 1.50 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
    • Analysis of 40 NI...
 details
UrineDetected and Quantified4-36 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified<15 umol/mmol creatinineNot SpecifiedNot SpecifiedNormal details
UrineDetected and Quantified11.2 (4.6-33.6) umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified<26.15 umol/mmol creatinineChildren (1-13 years old)Both
Normal
    • BC Children's Hos...
 details
UrineDetected and Quantified51.9 (13.7-97.3) umol/mmol creatinineNewborn (0-30 days old)BothNormal details
UrineDetected and Quantified41.5 (14.6-150.3) umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified12.4 (5.1-24.9) umol/mmol creatinineChildren (1-13 years old)BothNormal details
UrineDetected and Quantified11.2 (4.6-33.6) umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified24.379 umol/mmol creatinineChildren (1-13 years old)Not Specified
Normal
    • Analysis of 30 no...
 details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal		 details
UrineDetected and Quantified2.2 (0.8-52) umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified33.0 (13.3-77.9) umol/mmol creatinineAdult (>18 years old)Both
Normal		 details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.6 (0.30-0.90) uMAdult (>18 years old)BothD-2-hydroxyglutaric aciduria
    • MetaGene: Metabol...
 details
BloodDetected and Quantified61.0 +/- 14.0 uMAdult (>18 years old)BothD-2-Hydroxyglutaric aciduria details
BloodDetected and Quantified47.0 +/- 13.0 uMAdult (>18 years old)BothL-2-hydroxyglutaric aciduria details
Cerebrospinal Fluid (CSF)Detected and Quantified0.2 (0.1-0.30) uMAdult (>18 years old)BothD-2-hydroxyglutaric aciduria
    • MetaGene: Metabol...
 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer		 details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)MaleSupragingival Plaque details
UrineDetected and Quantified61.074 +/- 36.982 umol/mmol creatinineChildren (1-13 years old)Both
Eosinophilic esophagitis
    • Analysis of 30 no...
 details
UrineDetected and Quantified62 umol/mmol creatinineNewborn (0-30 days old)FemaleDeafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome details
UrineDetected and Quantified685 - 786 umol/mmol creatinineInfant (0-1 year old)MaleD-2-hydroxyglutaric aciduria details
UrineDetected and Quantified113 umol/mmol creatinineNewborn (0-30 days old)Male3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD) details
UrineDetected and Quantified4 umol/mmol creatinineAdult (>18 years old)Male
27-hydroxylase deficiency		 details
UrineDetected and Quantified630-1420 umol/mmol creatinineChildren (1-13 years old)BothL-2-hydroxyglutaric aciduria details
UrineDetected and Quantified33.0 (20.0-46.0) umol/mmol creatinineAdult (>18 years old)BothGlutaric aciduria II
    • MetaGene: Metabol...
 details
UrineDetected and Quantified2175.0 (50.0-4300.0) umol/mmol creatinineChildren (1-13 years old)BothGlutaric aciduria II
    • MetaGene: Metabol...
 details
UrineDetected and Quantified373 umol/mmol creatinineChildren (1-13 years old)MaleD, L-2-hydroxygluraric aciduria details
UrineDetected and Quantified10.0 (3.0-17.0) umol/mmol creatinineAdult (>18 years old)BothD-2-Hydroxyglutaric aciduria
    • MetaGene: Metabol...
 details
Associated Disorders and Diseases
Disease References
D-2-hydroxyglutaric aciduria
  1. Gibson KM, ten Brink HJ, Schor DS, Kok RM, Bootsma AH, Hoffmann GF, Jakobs C: Stable-isotope dilution analysis of D- and L-2-hydroxyglutaric acid: application to the detection and prenatal diagnosis of D- and L-2-hydroxyglutaric acidemias. Pediatr Res. 1993 Sep;34(3):277-80. [PubMed:8134166 ]
  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 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
L-2-Hydroxyglutaric aciduria
  1. Gibson KM, ten Brink HJ, Schor DS, Kok RM, Bootsma AH, Hoffmann GF, Jakobs C: Stable-isotope dilution analysis of D- and L-2-hydroxyglutaric acid: application to the detection and prenatal diagnosis of D- and L-2-hydroxyglutaric acidemias. Pediatr Res. 1993 Sep;34(3):277-80. [PubMed:8134166 ]
  2. Barbot C, Fineza I, Diogo L, Maia M, Melo J, Guimaraes A, Pires MM, Cardoso ML, Vilarinho L: L-2-Hydroxyglutaric aciduria: clinical, biochemical and magnetic resonance imaging in six Portuguese pediatric patients. Brain Dev. 1997 Jun;19(4):268-73. [PubMed:9187477 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Colorectal cancer
  1. 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 ]
  2. 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 ]
  3. 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 ]
Supragingival Plaque
  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 ]
Glutaric aciduria II
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
D, L-2-hydroxyglutaric aciduria
  1. Muntau AC, Roschinger W, Merkenschlager A, van der Knaap MS, Jakobs C, Duran M, Hoffmann GF, Roscher AA: Combined D-2- and L-2-hydroxyglutaric aciduria with neonatal onset encephalopathy: a third biochemical variant of 2-hydroxyglutaric aciduria? Neuropediatrics. 2000 Jun;31(3):137-40. doi: 10.1055/s-2000-7497. [PubMed:10963100 ]
  2. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Cerebrotendinous xanthomatosis
  1. Siman-Tov T, Meiner V, Gadoth N: Could steroids mask the diagnosis of cerebrotendinous xanthomatosis? J Neurol Sci. 2006 Apr 15;243(1-2):83-6. Epub 2006 Jan 30. [PubMed:16445943 ]
3-Hydroxyacyl-CoA dehydrogenase deficiency
  1. Vilarinho L, Marques JS, Rocha H, Ramos A, Lopes L, Narayan SB, Bennett MJ: Diagnosis of a patient with a kinetic variant of medium and short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency by newborn screening. Mol Genet Metab. 2012 Jul;106(3):277-80. doi: 10.1016/j.ymgme.2012.04.005. Epub 2012 Apr 13. [PubMed:22579592 ]
Deafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome
  1. James AW, Miranda SG, Culver K, Hall BD, Golabi M: DOOR syndrome: clinical report, literature review and discussion of natural history. Am J Med Genet A. 2007 Dec 1;143A(23):2821-31. doi: 10.1002/ajmg.a.32054. [PubMed:17994565 ]
Associated OMIM IDs
  • 600721 (D-2-hydroxyglutaric aciduria)
  • 236792 (L-2-Hydroxyglutaric aciduria)
  • 114500 (Colorectal cancer)
  • 231680 (Glutaric aciduria II)
  • 610247 (Eosinophilic esophagitis)
  • 615182 (D, L-2-hydroxyglutaric aciduria)
  • 213700 (Cerebrotendinous xanthomatosis)
  • 231530 (3-Hydroxyacyl-CoA dehydrogenase deficiency)
  • 220500 (Deafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDNot Available
KNApSAcK IDNot Available
Chemspider ID42
KEGG Compound IDC02630
BioCyc ID2-HYDROXYGLUTARIC_ACID
BiGG IDNot Available
Wikipedia LinkAlpha-Hydroxyglutaric_acid
METLIN IDNot Available
PubChem Compound43
PDB IDNot Available
ChEBI ID17084
Food Biomarker OntologyNot Available
VMH IDM00653
MarkerDB IDMDB00029834
Good Scents IDNot Available
References
Synthesis ReferenceKobayashi, Hidehiko; Yamaguchi, Koretaka; Yamashita, Takeshi. a-Hydroxyglutaric acid from glutamic acid. Jpn. Tokkyo Koho (1968), 3 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. 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 ]
  2. Gibson KM, ten Brink HJ, Schor DS, Kok RM, Bootsma AH, Hoffmann GF, Jakobs C: Stable-isotope dilution analysis of D- and L-2-hydroxyglutaric acid: application to the detection and prenatal diagnosis of D- and L-2-hydroxyglutaric acidemias. Pediatr Res. 1993 Sep;34(3):277-80. [PubMed:8134166 ]
  3. van der Knaap MS, Jakobs C, Hoffmann GF, Nyhan WL, Renier WO, Smeitink JA, Catsman-Berrevoets CE, Hjalmarson O, Vallance H, Sugita K, Bowe CM, Herrin JT, Craigen WJ, Buist NR, Brookfield DS, Chalmers RA: D-2-Hydroxyglutaric aciduria: biochemical marker or clinical disease entity? Ann Neurol. 1999 Jan;45(1):111-9. [PubMed:9894884 ]
  4. Rashed MS, AlAmoudi M, Aboul-Enein HY: Chiral liquid chromatography tandem mass spectrometry in the determination of the configuration of 2-hydroxyglutaric acid in urine. Biomed Chromatogr. 2000 Aug;14(5):317-20. [PubMed:10960831 ]
  5. da Silva CG, Bueno AR, Schuck PF, Leipnitz G, Ribeiro CA, Wannmacher CM, Wyse AT, Wajner M: D-2-hydroxyglutaric acid inhibits creatine kinase activity from cardiac and skeletal muscle of young rats. Eur J Clin Invest. 2003 Oct;33(10):840-7. [PubMed:14511354 ]
  6. Clarke NF, Andrews I, Carpenter K, Jakobs C, van der Knaap MS, Kirk EP: D-2-hydroxyglutaric aciduria: a case with an intermediate phenotype and prenatal diagnosis of two affected fetuses. Am J Med Genet A. 2003 Aug 1;120A(4):523-7. [PubMed:12884432 ]
  7. Fujitake J, Ishikawa Y, Fujii H, Nishimura K, Hayakawa K, Inoue F, Terada N, Okochi M, Tatsuoka Y: L-2-hydroxyglutaric aciduria: two Japanese adult cases in one family. J Neurol. 1999 May;246(5):378-82. [PubMed:10399870 ]
  8. Barth PG, Hoffmann GF, Jaeken J, Lehnert W, Hanefeld F, van Gennip AH, Duran M, Valk J, Schutgens RB, Trefz FK, et al.: L-2-hydroxyglutaric acidemia: a novel inherited neurometabolic disease. Ann Neurol. 1992 Jul;32(1):66-71. [PubMed:1642474 ]
  9. de Klerk JB, Huijmans JG, Stroink H, Robben SG, Jakobs C, Duran M: L-2-hydroxyglutaric aciduria: clinical heterogeneity versus biochemical homogeneity in a sibship. Neuropediatrics. 1997 Dec;28(6):314-7. [PubMed:9453028 ]
  10. Bleiweis AS, Reeves HC, Ajl SJ: Formation of alpha-hydroxyglutaric acid by Aspergillus glaucus. J Bacteriol. 1967 Nov;94(5):1560-4. [PubMed:6057807 ]

Enzymes

Enzyme Details
1. D-3-phosphoglycerate dehydrogenase
General function:
Involved in phosphoglycerate dehydrogenase activity
Specific function:
Not Available
Gene Name:
PHGDH
Uniprot ID:
O43175
Molecular weight:
56650.03
Reactions
2-Hydroxyglutarate + NAD → Oxoglutaric acid + NADHdetails
Enzyme Details
2. D-2-hydroxyglutarate dehydrogenase, mitochondrial
General function:
Involved in catalytic activity
Specific function:
Catalyzes the oxidation of D-2-hydroxyglutarate to alpha-ketoglutarate.
Gene Name:
D2HGDH
Uniprot ID:
Q8N465
Molecular weight:
56415.58
Enzyme Details
3. L-2-hydroxyglutarate dehydrogenase, mitochondrial
General function:
Involved in 2-hydroxyglutarate dehydrogenase activity
Specific function:
Not Available
Gene Name:
L2HGDH
Uniprot ID:
Q9H9P8
Molecular weight:
50315.58
Reactions
2-Hydroxyglutarate + FAD → Oxoglutaric acid + FADHdetails
Enzyme Details
4. Hydroxyacid-oxoacid transhydrogenase, mitochondrial
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA) coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG). D,L-3-hydroxyisobutyrate and L-3-hydroxybutyrate (L-3-OHB) are also substrates for HOT with 10-fold lower activities.
Gene Name:
ADHFE1
Uniprot ID:
Q8IWW8
Molecular weight:
50307.42