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Showing metabocard for D-2-Hydroxyglutaric acid (HMDB0000606)

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enzymes (3) Show 3 proteins
Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-02-21 17:14:59 UTC
HMDB IDHMDB0000606
Secondary Accession Numbers
  • HMDB0002323
  • HMDB00606
  • HMDB02323
Metabolite Identification
Common NameD-2-Hydroxyglutaric acid
DescriptionIn humans, D-2-hydroxyglutaric acid is formed by a hydroxyacid-oxoacid transhydrogenase whereas in bacteria it is formed by a 2-hydroxyglutarate synthase. D-2-Hydroxyglutaric acid is also formed via the normal activity of hydroxyacid-oxoacid transhydrogenase during conversion of 4-hydroxybutyrate to succinate semialdehyde. The compound can be converted to alpha-ketoglutaric acid through the action of a 2-hydroxyglutarate dehydrogenase (EC 1.1.99.2). In humans, there are two such enzymes (D2HGDH and L2HGDH). Both the D and the L stereoisomers of hydroxyglutaric acid are found in body fluids. D-2-Hydroxyglutaric acid is a biochemical hallmark of the inherited neurometabolic disorder D-2-hydroxyglutaric aciduria (OMIM: 600721 ) and the genetic disorder glutaric aciduria II. 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. An elevated urine level of D-2-hydroxyglutaric acid has been reported in patients with spondyloenchondrodysplasia (OMIM: 271550 ). D-2-Hydroxyglutaric acid can be converted to alpha-ketoglutaric acid through the action of 2-hydroxyglutarate dehydrogenase (D2HGDH). Additionally, the enzyme D-3-phosphoglycerate dehydrogenase (PHGDH) can catalyze the NADH-dependent reduction of alpha-ketoglutarate (AKG) to D-2-hydroxyglutarate (D-2HG). Nyhan et al. (1995) described 3 female patients, 2 of them sibs, who were found to have excess accumulation of D-2-hydroxyglutaric acid in the urine. The phenotype was quite variable, even among the sibs, but included mental retardation, macrocephaly with cerebral atrophy, hypotonia, seizures, and involuntary movements. One of the patients developed severe intermittent vomiting and was given a pyloromyotomy. The electroencephalogram demonstrated hypsarrhythmia. There was an increased concentration of protein in cerebrospinal fluid, an unusual finding in inborn errors of metabolism. D-2-Hydroxyglutaric acid can also be produced via gain-of-function mutations in the cytosolic and mitochondrial isoforms of isocitrate dehydrogenase (IDH). IDH is part of the TCA cycle and this compound is generated in high abundance when IDH is mutated. Since D-2-hydroxyglutaric acid is sufficiently similar in structure to 2-oxoglutarate (2OG), 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-inducible factor (HIF)-mediated hypoxic response and alterations in gene expression through global epigenetic remodeling. The net effect is that D-2-hydroxyglutaric acid causes a cascading effect that leads genetic perturbations and malignant transformation. Depending on the circumstances, D-2-hydroxyglutaric acid can act as an oncometabolite, a neurotoxin, an acidogen, and a metabotoxin. An oncometabolite is a compound that promotes tumour growth and survival. A neurotoxin is compound that is toxic to neurons or nerual tissue. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. As an oncometabolite, D-2-hydroxyglutaric acid is a competitive inhibitor of multiple alpha-ketoglutarate-dependent dioxygenases, including histone demethylases and the TET family of 5mC hydroxylases. As a result, high levels of 2-hydroxyglutarate lead to genome-wide histone and DNA methylation alterations, which in turn lead to mutations that ultimately cause cancer (PMID: 29038145 ). As a neurotoxin, D-2-hydroxyglutaric acid mediates its neurotoxicity through activation of N-methyl-D-aspartate receptors. D-2-Hydroxyglutaric acid is structurally similar to the excitatory amino acid glutamate and stimulates neurodegeneration by mechanisms similar to glutamate, NMDA, or mitochondrial toxins (PMID: 12153528 ). As an acidogen, D-2-hydroxyglutaric acid is classified as an alpha hydroxy acid belonging to the general class of compounds known as organic acids. Chronically high levels of D-2-hydroxyglutaric acid are a feature of the inborn error of metabolism called D-2-hydroxyglutaric aciduria. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, kidney abnormalities, liver damage, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated D-2-hydroxyglutaric aciduria. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures.
Structure
Data?1676999699
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0000606 (D-2-Hydroxyglutaric acid)


  Mrv1652309042000042D          

 10  9  0  0  0  0            999 V2000
 9998.6124 9997.7078    0.0000 C   0  0  2  0  0  0  0  0  0  0  0  0
 9997.8979 9998.1199    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9997.1831 9997.7078    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9996.4684 9998.1199    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9995.7534 9997.7078    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9996.4684 9998.9448    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9999.3301 9998.1199    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10000.0437 9997.7078    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9999.3301 9998.9448    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.6124 9996.8823    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  6  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 HMDB0000606 (D-2-Hydroxyglutaric acid)

HMDB0000606
     RDKit          3D
D-2-Hydroxyglutaric acid
 18 17  0  0  0  0  0  0  0  0999 V2000
    2.0071   -1.4622   -0.8367 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.2168   -0.3952   -0.2314 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.3637    0.3277   -0.5408 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.2908    0.1247    0.7998 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0001    0.6475    0.1814 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7330   -0.4413   -0.5773 C   0  0  1  0  0  0  0  0  0  0  0  0
   -1.0378   -1.4697    0.3174 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9852    0.1567   -1.0954 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9301    1.1038   -1.9169 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1952   -0.3186   -0.6827 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.3322    1.3316   -0.7178 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.7715    0.9567    1.3523 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.0250   -0.6823    1.5258 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6727    1.0107    0.9645 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.2510    1.5061   -0.4749 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1579   -0.8307   -1.4327 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9996   -1.4842    0.5308 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5465   -0.0812    0.2523 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  6
  7 17  1  0
 10 18  1  0
M  END
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3D SDF for HMDB0000606 (D-2-Hydroxyglutaric acid)


  Mrv1652309042000042D          

 10  9  0  0  0  0            999 V2000
 9998.6124 9997.7078    0.0000 C   0  0  2  0  0  0  0  0  0  0  0  0
 9997.8979 9998.1199    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9997.1831 9997.7078    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9996.4684 9998.1199    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9995.7534 9997.7078    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9996.4684 9998.9448    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9999.3301 9998.1199    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10000.0437 9997.7078    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9999.3301 9998.9448    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.6124 9996.8823    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  6  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>
HMDB0000606

> <DATABASE_NAME>
hmdb

> <SMILES>
O[C@H](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)/t3-/m1/s1

> <INCHI_KEY>
HWXBTNAVRSUOJR-GSVOUGTGSA-N

> <FORMULA>
C5H8O5

> <MOLECULAR_WEIGHT>
148.114

> <EXACT_MASS>
148.037173366

> <JCHEM_ACCEPTOR_COUNT>
5

> <JCHEM_ATOM_COUNT>
18

> <JCHEM_AVERAGE_POLARIZABILITY>
12.972875741537502

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
3

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
(2R)-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>
3.276485914460607

> <JCHEM_PKA_STRONGEST_BASIC>
-3.8068384384876675

> <JCHEM_POLAR_SURFACE_AREA>
94.83

> <JCHEM_REFRACTIVITY>
29.6302

> <JCHEM_ROTATABLE_BOND_COUNT>
4

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
1.57e+02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
D-2-hydroxyglutaric acid

> <JCHEM_VEBER_RULE>
0

$$$$
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3D-SDF for HMDB0000606 (D-2-Hydroxyglutaric acid)

HMDB0000606
     RDKit          3D
D-2-Hydroxyglutaric acid
 18 17  0  0  0  0  0  0  0  0999 V2000
    2.0071   -1.4622   -0.8367 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.2168   -0.3952   -0.2314 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.3637    0.3277   -0.5408 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.2908    0.1247    0.7998 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0001    0.6475    0.1814 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7330   -0.4413   -0.5773 C   0  0  1  0  0  0  0  0  0  0  0  0
   -1.0378   -1.4697    0.3174 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9852    0.1567   -1.0954 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9301    1.1038   -1.9169 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1952   -0.3186   -0.6827 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.3322    1.3316   -0.7178 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.7715    0.9567    1.3523 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.0250   -0.6823    1.5258 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6727    1.0107    0.9645 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.2510    1.5061   -0.4749 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1579   -0.8307   -1.4327 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9996   -1.4842    0.5308 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5465   -0.0812    0.2523 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  6
  7 17  1  0
 10 18  1  0
M  END
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PDB for HMDB0000606 (D-2-Hydroxyglutaric acid)

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    8664.0768662.388   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0    8662.7438663.157   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0    8661.4088662.388   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0    8660.0748663.157   0.000  0.00  0.00           C+0
HETATM    5  O   UNK     0    8658.7408662.388   0.000  0.00  0.00           O+0
HETATM    6  O   UNK     0    8660.0748664.697   0.000  0.00  0.00           O+0
HETATM    7  C   UNK     0    8665.4168663.157   0.000  0.00  0.00           C+0
HETATM    8  O   UNK     0    8666.7488662.388   0.000  0.00  0.00           O+0
HETATM    9  O   UNK     0    8665.4168664.697   0.000  0.00  0.00           O+0
HETATM   10  O   UNK     0    8664.0768660.847   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 HMDB0000606 (D-2-Hydroxyglutaric acid)

COMPND    HMDB0000606
HETATM    1  O1  UNL     1       2.007  -1.462  -0.837  1.00  0.00           O  
HETATM    2  C1  UNL     1       2.217  -0.395  -0.231  1.00  0.00           C  
HETATM    3  O2  UNL     1       3.364   0.328  -0.541  1.00  0.00           O  
HETATM    4  C2  UNL     1       1.291   0.125   0.800  1.00  0.00           C  
HETATM    5  C3  UNL     1      -0.000   0.648   0.181  1.00  0.00           C  
HETATM    6  C4  UNL     1      -0.733  -0.441  -0.577  1.00  0.00           C  
HETATM    7  O3  UNL     1      -1.038  -1.470   0.317  1.00  0.00           O  
HETATM    8  C5  UNL     1      -1.985   0.157  -1.095  1.00  0.00           C  
HETATM    9  O4  UNL     1      -1.930   1.104  -1.917  1.00  0.00           O  
HETATM   10  O5  UNL     1      -3.195  -0.319  -0.683  1.00  0.00           O  
HETATM   11  H1  UNL     1       3.332   1.332  -0.718  1.00  0.00           H  
HETATM   12  H2  UNL     1       1.772   0.957   1.352  1.00  0.00           H  
HETATM   13  H3  UNL     1       1.025  -0.682   1.526  1.00  0.00           H  
HETATM   14  H4  UNL     1      -0.673   1.011   0.965  1.00  0.00           H  
HETATM   15  H5  UNL     1       0.251   1.506  -0.475  1.00  0.00           H  
HETATM   16  H6  UNL     1      -0.158  -0.831  -1.433  1.00  0.00           H  
HETATM   17  H7  UNL     1      -2.000  -1.484   0.531  1.00  0.00           H  
HETATM   18  H8  UNL     1      -3.547  -0.081   0.252  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 HMDB0000606 (D-2-Hydroxyglutaric acid)

O[C@H](CCC(O)=O)C(O)=O
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INCHI for HMDB0000606 (D-2-Hydroxyglutaric acid)

InChI=1S/C5H8O5/c6-3(5(9)10)1-2-4(7)8/h3,6H,1-2H2,(H,7,8)(H,9,10)/t3-/m1/s1
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View in JSmolView NMR AssignmentsView Stereo Labels
Synonyms
ValueSource
(R)-2-HydroxyglutarateChEBI
(R)-HydroxyglutarateChEBI
(R)-2-Hydroxyglutaric acidGenerator
(R)-Hydroxyglutaric acidGenerator
D-2-HydroxyglutarateGenerator
(R)-2-Hydroxy-pentanedioateHMDB
(R)-2-Hydroxy-pentanedioic acidHMDB
(R)-a-HydroxyglutarateHMDB
(R)-a-Hydroxyglutaric acidHMDB
(R)-alpha-HydroxyglutarateHMDB
(R)-alpha-Hydroxyglutaric acidHMDB
2-Hydroxy-D-glutarateHMDB
2-Hydroxy-D-glutaric acidHMDB
2-Hydroxy-delta-glutarateHMDB
2-Hydroxy-delta-glutaric acidHMDB
D-a-HydroxyglutarateHMDB
D-a-Hydroxyglutaric acidHMDB
delta-2-HydroxyglutarateHMDB
delta-2-Hydroxyglutaric acidHMDB
delta-alpha-HydroxyglutarateHMDB
delta-alpha-Hydroxyglutaric acidHMDB
2-HydroxyglutarateHMDB
alpha-Hydroxyglutarate, (D)-isomerHMDB
alpha-Hydroxyglutarate, (DL)-isomerHMDB
2-Hydroxyglutaric acidHMDB
alpha-HydroxyglutarateHMDB
alpha-Hydroxyglutarate, (L)-isomerHMDB
alpha-Hydroxyglutarate, disodium saltHMDB
Chemical FormulaC5H8O5
Average Molecular Weight148.114
Monoisotopic Molecular Weight148.037173366
IUPAC Name(2R)-2-hydroxypentanedioic acid
Traditional NameD-2-hydroxyglutaric acid
CAS Registry Number13095-47-1
SMILES
O[C@H](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)/t3-/m1/s1
InChI KeyHWXBTNAVRSUOJR-GSVOUGTGSA-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 effect
Health effect
Disposition
Biological locationSource
Process
Naturally occurring process
Role
Indirect biological roleBiological roleIndustrial application
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point> 300 °CNot 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]+133.04831661259
DarkChem[M+H]+133.04831661259
DarkChem[M-H]-125.30531661259
DarkChem[M-H]-125.30531661259
AllCCS[M+H]+134.03432859911
AllCCS[M-H]-126.53232859911
DeepCCS[M+H]+126.79330932474
DeepCCS[M-H]-122.92730932474
DeepCCS[M-2H]-160.49530932474
DeepCCS[M+Na]+135.8830932474
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
D-2-Hydroxyglutaric acidO[C@H](CCC(O)=O)C(O)=O2340.9Standard polar33892256
D-2-Hydroxyglutaric acidO[C@H](CCC(O)=O)C(O)=O1100.2Standard non polar33892256
D-2-Hydroxyglutaric acidO[C@H](CCC(O)=O)C(O)=O1428.9Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
D-2-Hydroxyglutaric acid,1TMS,isomer #1C[Si](C)(C)O[C@H](CCC(=O)O)C(=O)O1441.3Semi standard non polar33892256
D-2-Hydroxyglutaric acid,1TMS,isomer #2C[Si](C)(C)OC(=O)CC[C@@H](O)C(=O)O1442.3Semi standard non polar33892256
D-2-Hydroxyglutaric acid,1TMS,isomer #3C[Si](C)(C)OC(=O)[C@H](O)CCC(=O)O1400.3Semi standard non polar33892256
D-2-Hydroxyglutaric acid,2TMS,isomer #1C[Si](C)(C)OC(=O)CC[C@@H](O[Si](C)(C)C)C(=O)O1536.2Semi standard non polar33892256
D-2-Hydroxyglutaric acid,2TMS,isomer #2C[Si](C)(C)OC(=O)[C@@H](CCC(=O)O)O[Si](C)(C)C1504.0Semi standard non polar33892256
D-2-Hydroxyglutaric acid,2TMS,isomer #3C[Si](C)(C)OC(=O)CC[C@@H](O)C(=O)O[Si](C)(C)C1476.7Semi standard non polar33892256
D-2-Hydroxyglutaric acid,3TMS,isomer #1C[Si](C)(C)OC(=O)CC[C@@H](O[Si](C)(C)C)C(=O)O[Si](C)(C)C1584.8Semi standard non polar33892256
D-2-Hydroxyglutaric acid,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)O[C@H](CCC(=O)O)C(=O)O1698.7Semi standard non polar33892256
D-2-Hydroxyglutaric acid,1TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)CC[C@@H](O)C(=O)O1702.2Semi standard non polar33892256
D-2-Hydroxyglutaric acid,1TBDMS,isomer #3CC(C)(C)[Si](C)(C)OC(=O)[C@H](O)CCC(=O)O1672.0Semi standard non polar33892256
D-2-Hydroxyglutaric acid,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CC[C@@H](O[Si](C)(C)C(C)(C)C)C(=O)O1992.1Semi standard non polar33892256
D-2-Hydroxyglutaric acid,2TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC(=O)[C@@H](CCC(=O)O)O[Si](C)(C)C(C)(C)C1979.7Semi standard non polar33892256
D-2-Hydroxyglutaric acid,2TBDMS,isomer #3CC(C)(C)[Si](C)(C)OC(=O)CC[C@@H](O)C(=O)O[Si](C)(C)C(C)(C)C1935.0Semi standard non polar33892256
D-2-Hydroxyglutaric acid,3TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CC[C@@H](O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C2222.5Semi standard non polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (Non-derivatized) - 70eV, Positivesplash10-0zgl-9300000000-805667744aba36281cec2017-09-01Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (3 TMS) - 70eV, Positivesplash10-00gv-8191000000-f6fdffd87ffe1acf65b42017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (TMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (TMS_1_3) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (TMS_2_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (TMS_2_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (TMS_2_3) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (TBDMS_1_3) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (TBDMS_2_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (TBDMS_2_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (TBDMS_2_3) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - D-2-Hydroxyglutaric acid GC-MS (TBDMS_3_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - D-2-Hydroxyglutaric acid 10V, Positive-QTOFsplash10-001j-1900000000-dbf80fc2b86ec60d623e2016-06-03Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - D-2-Hydroxyglutaric acid 20V, Positive-QTOFsplash10-0m5r-9600000000-d1bc7ce2206164b5bc2c2016-06-03Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - D-2-Hydroxyglutaric acid 40V, Positive-QTOFsplash10-0a4i-9000000000-df3e77cb42d6f7647a642016-06-03Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - D-2-Hydroxyglutaric acid 10V, Negative-QTOFsplash10-0002-1900000000-095c1140772d4cdd149b2016-08-03Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - D-2-Hydroxyglutaric acid 20V, Negative-QTOFsplash10-0zfr-7900000000-ce158ac5b56eff8677352016-08-03Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - D-2-Hydroxyglutaric acid 40V, Negative-QTOFsplash10-0a4i-9000000000-e30760384709aa9789862016-08-03Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - D-2-Hydroxyglutaric acid 10V, Negative-QTOFsplash10-0fbi-3900000000-f88fcf3ac0dd06636b162021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - D-2-Hydroxyglutaric acid 20V, Negative-QTOFsplash10-0k9i-9400000000-31bf8d3bb7203e7bf2002021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - D-2-Hydroxyglutaric acid 40V, Negative-QTOFsplash10-0a4u-9000000000-c0ac45c4d66178ac20cf2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - D-2-Hydroxyglutaric acid 10V, Positive-QTOFsplash10-0w39-4900000000-1385b471f7cdf14ca14d2021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - D-2-Hydroxyglutaric acid 20V, Positive-QTOFsplash10-052r-9500000000-17ece6956644d4cf20692021-09-22Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - D-2-Hydroxyglutaric acid 40V, Positive-QTOFsplash10-0a4r-9000000000-dbb4195080bddb5f62102021-09-22Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum

IR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)2023-02-03FELIX labView Spectrum
Predicted IR SpectrumIR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)2023-02-03FELIX labView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Brain
  • Placenta
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified0.280-0.930 uMInfant (0-1 year old)MaleNormal details
BloodDetected and Quantified0.3-0.9 uMInfant (0-1 year old)BothNormal details
BloodDetected and Quantified0.7(0.3-0.9) uMInfant (0-1 year old)Female
Normal		 details
BloodDetected and Quantified0.7 +/- 0.2 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.07-0.3 uMInfant (0-1 year old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified0.0670-0.340 uMInfant (0-1 year old)MaleNormal details
FecesDetected but not QuantifiedNot QuantifiedNot SpecifiedNot Specified
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
UrineDetected and Quantified11.2 (4.6-33.6) umol/mmol creatinineAdolescent (13-18 years old)BothNormal details
UrineDetected and Quantified2.8-17 umol/mmol creatinineInfant (0-1 year old)MaleNormal details
UrineDetected and Quantified1.5-2.1 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified<15.267 umol/mmol creatinineChildren (1 - 13 years old)MaleNormal details
UrineDetected and Quantified0.34 +/- 0.16 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal details
UrineDetected and Quantified2.8-17 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified6(2.8-17) umol/mmol creatinineInfant (0-1 year old)Female
Normal		 details
UrineDetected and Quantified<26.15 umol/mmol creatinineChildren (1 - 18 years old)Both
Normal
    • BC Children's Hos...
 details
UrineDetected and Quantified1.0-1.8 umol/mmol creatinineAdult (>18 years old)MaleNormal 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
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified217 uMInfant (0-1 year old)MaleD-2-hydroxyglutaric aciduria details
BloodDetected and Quantified2.6(2.2-3.2) uMInfant (0-1 year old)BothCombined D,L-2-hydroxyglutaric aciduria details
BloodDetected and Quantified366(99-757) uMInfant (0-1 year old)Female
D-2-Hydroxyglutaric aciduria II		 details
BloodDetected and Quantified68(26-123) uMInfant (0-1 year old)Female
D-2-Hydroxyglutaric aciduria I		 details
BloodDetected and Quantified68(26-123) uMInfant (0-1 year old)Female
D-2-hydroxyglutaric aciduria type I		 details
BloodDetected and Quantified61.0 +/- 14.0 uMAdult (>18 years old)BothD-2-Hydroxyglutaric aciduria details
Cerebrospinal Fluid (CSF)Detected and Quantified1.1(0.42-2.5) uMInfant (0-1 year old)BothCombined D,L-2-hydroxyglutaric aciduria details
Cerebrospinal Fluid (CSF)Detected and Quantified61 uMInfant (0-1 year old)MaleD-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
UrineDetected but not QuantifiedNot QuantifiedChildren (1-13 years old)FemaleSpondyloenchondrodysplasia details
UrineDetected and Quantified228 umol/mmol creatinineChildren (1-13 years old)Male
D, L-2-hydroxygluraric aciduria		 details
UrineDetected and Quantified4067 umol/mmol creatinineInfant (0-1 year old)MaleD-2-hydroxyglutaric aciduria details
UrineDetected and Quantified97 umol/mmol creatinineInfant (0-1 year old)Female
D2-/L2-hydroxyglutaric aciduria		 details
UrineDetected and Quantified360 umol/mmol creatinineChildren (1 - 13 years old)MaleD-2-Hydroxyglutaric aciduria details
UrineDetected and Quantified427 umol/mmol creatinineInfant (0-1 year old)FemaleD2-/L2-hydroxyglutaric aciduria details
UrineDetected and Quantified632 umol/mmol creatinineInfant (0-1 year old)Female
D,L-2-hydroxyglutaric aciduria type I		 details
UrineDetected and Quantified915.997 umol/mmol creatinineChildren (1 - 13 years old)MaleD-2-Hydroxyglutaric aciduria details
UrineDetected and Quantified1511.395 umol/mmol creatinineChildren (1 - 13 years old)MaleD-2-Hydroxyglutaric aciduria details
UrineDetected and Quantified2486(448-11305) umol/mmol creatinineInfant (0-1 year old)Female
D-2-hydroxyglutaric aciduria type II		 details
UrineDetected and Quantified496(228-750) umol/mmol creatinineInfant (0-1 year old)BothCombined D,L-2-hydroxyglutaric 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. 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 ]
  3. Geerts Y, Renier WO, Bakkeren J, de Jong J: 2-Hydroxyglutaric aciduria: a case report on an infant with the D-isomeric form with review of the literature. J Neurol Sci. 1996 Nov;143(1-2):166-9. [PubMed:8981317 ]
  4. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
D, L-2-hydroxyglutaric aciduria
  1. Kranendijk M, Struys EA, Salomons GS, Van der Knaap MS, Jakobs C: Progress in understanding 2-hydroxyglutaric acidurias. J Inherit Metab Dis. 2012 Jul;35(4):571-87. doi: 10.1007/s10545-012-9462-5. Epub 2012 Mar 6. [PubMed:22391998 ]
  2. 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 ]
  3. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
Spondyloenchondrodysplasia
  1. Talkhani IS, Saklatvala J, Dwyer J: D-2-hydroxyglutaric aciduria in association with spondyloenchondromatosis. Skeletal Radiol. 2000 May;29(5):289-92. [PubMed:10883451 ]
Associated OMIM IDs
  • 600721 (D-2-hydroxyglutaric aciduria)
  • 615182 (D, L-2-hydroxyglutaric aciduria)
  • 271550 (Spondyloenchondrodysplasia)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022139
KNApSAcK IDNot Available
Chemspider ID388508
KEGG Compound IDC01087
BioCyc IDR-2-HYDROXYGLUTARATE
BiGG IDNot Available
Wikipedia LinkAlpha-Hydroxyglutaric acid
METLIN ID5581
PubChem Compound439391
PDB IDNot Available
ChEBI ID32796
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDMDB00000198
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. 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. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 ]
  6. Collins RRJ, Patel K, Putnam WC, Kapur P, Rakheja D: Oncometabolites: A New Paradigm for Oncology, Metabolism, and the Clinical Laboratory. Clin Chem. 2017 Dec;63(12):1812-1820. doi: 10.1373/clinchem.2016.267666. Epub 2017 Oct 16. [PubMed:29038145 ]
  7. Kolker S, Pawlak V, Ahlemeyer B, Okun JG, Horster F, Mayatepek E, Krieglstein J, Hoffmann GF, Kohr G: NMDA receptor activation and respiratory chain complex V inhibition contribute to neurodegeneration in d-2-hydroxyglutaric aciduria. Eur J Neurosci. 2002 Jul;16(1):21-8. [PubMed:12153528 ]
  8. 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 ]

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
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
Reactions
D-2-Hydroxyglutaric acid + acceptor → Oxoglutaric acid + reduced acceptordetails
Enzyme Details
3. 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
Reactions
(S)-3-Hydroxybutyric acid + Oxoglutaric acid → Acetoacetic acid + D-2-Hydroxyglutaric aciddetails
4-Hydroxybutyric acid + Oxoglutaric acid → Succinic acid semialdehyde + D-2-Hydroxyglutaric aciddetails