MiMeDB: Showing metabocard for 2-Hydroxyglutarate (MMDBc0000675)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-12-10 18:52:29 UTC

Update Date

2022-08-31 06:10:51 UTC

Metabolite ID

MMDBc0000675

Metabolite Identification

Common Name

2-Hydroxyglutarate

Description

2-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

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

Synonyms

Value	Source
alpha-Hydroxyglutaric acid	ChEBI
alpha-Hydroxyglutarate	Kegg
a-Hydroxyglutarate	Generator
a-Hydroxyglutaric acid	Generator
Α-hydroxyglutarate	Generator
Α-hydroxyglutaric acid	Generator
2-Hydroxyglutaric acid	Generator
D-2-Hydroxyglutarate	HMDB
alpha-Hydroxyglutarate, (D)-isomer	HMDB
alpha-Hydroxyglutarate, (DL)-isomer	HMDB
alpha-Hydroxyglutarate, (L)-isomer	HMDB
alpha-Hydroxyglutarate, disodium salt	HMDB
(±)-2-hydroxyglutaric acid	HMDB
2,3-Dideoxypentaric acid	HMDB
2-Hydroxypentanedioic acid	HMDB
DL-2-Hydroxyglutaric acid	HMDB
2-Hydroxyglutarate	Generator

Molecular Formula

C₅H₈O₅

Average Mass

148.114

Monoisotopic Mass

148.037173366

IUPAC Name

2-hydroxypentanedioic acid

Traditional Name

2-hydroxyglutaric acid

CAS Registry Number

Not Available

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

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.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Hydroxy acids and derivatives

Sub Class

Short-chain hydroxy acids and derivatives

Direct Parent

Short-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 Framework

Aliphatic acyclic compounds

External Descriptors

2-hydroxydicarboxylic acid (CHEBI:17084 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Property	Value	Source
Water Solubility	157 g/L	ALOGPS
logP	-1	ALOGPS
logP	-0.82	ChemAxon
logS	0.03	ALOGPS
pKa (Strongest Acidic)	3.28	ChemAxon
pKa (Strongest Basic)	-3.8	ChemAxon
Physiological Charge	-2	ChemAxon
Hydrogen Acceptor Count	5	ChemAxon
Hydrogen Donor Count	3	ChemAxon
Polar Surface Area	94.83 Å²	ChemAxon
Rotatable Bond Count	4	ChemAxon
Refractivity	29.63 m³·mol⁻¹	ChemAxon
Polarizability	12.97 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Spectra

Spectrum Type	Description	Splash Key	Deposition Date	View
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	splash10-0uel-9300000000-fbe125fbbc3b5bc0783b	2016-09-22	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (3 TMS) - 70eV, Positive	splash10-00gv-8191000000-f6fdffd87ffe1acf65b4	2017-10-06	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 0V, negative	splash10-0002-0900000000-09163ea46c418972472c	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 1V, negative	splash10-0002-0900000000-d04b995445fdf4b9a53d	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 1V, negative	splash10-0002-0900000000-b0a25d5d220611188e25	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 1V, negative	splash10-0002-0900000000-fffbc099f0f67f0511f3	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 2V, negative	splash10-0002-0900000000-09f10084fba5e519de0a	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 2V, negative	splash10-002b-0900000000-dff435b3dcd956320479	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 3V, negative	splash10-004j-0900000000-bda2b170e24c7a9e5c35	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 3V, negative	splash10-004i-0900000000-73ae87a8fb9bc7ee539d	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 4V, negative	splash10-0fb9-1900000000-b1dc0a848bf8324490a7	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 5V, negative	splash10-0ug0-3900000000-07793b211b4728e17669	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 6V, negative	splash10-0udr-6900000000-f034f6e5155f6da3db5a	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - n/a 10V, negative	splash10-004i-0900000000-80c3e765d7538561ccbf	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - n/a 10V, negative	splash10-000i-9300000000-7ffbc20d629772e358cc	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 1V, negative	splash10-004i-0900000000-982cc580a7d58e352fcb	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 1V, negative	splash10-004i-1900000000-5920ffef63672e32cc73	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 2V, negative	splash10-004i-2900000000-0945bfdbb47d1208ca55	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 2V, negative	splash10-004r-3900000000-8a092cb937763eb031bb	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 3V, negative	splash10-004r-6900000000-41fa2c2ee20825e5b11d	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 4V, negative	splash10-002r-9800000000-6677f366953c5f35a17b	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 4V, negative	splash10-000i-9500000000-0ca597332561395d2cc3	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 5V, negative	splash10-000i-9400000000-510a9566cf27424fa58c	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - n/a 10V, negative	splash10-0udr-4900000000-cf48fcc4c8561ba5a0e0	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - LC-ESI-QTOF 35V, positive	splash10-00di-9000000000-36768b96f759cfee016d	2020-07-21	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - n/a 10V, positive	splash10-0udi-0900000000-47e5cb48d7b683b18747	2020-07-22	View Spectrum
LC-MS/MS	LC-MS/MS Spectrum - Orbitrap 1V, positive	splash10-03dj-1790000000-8eb0579a5917747d340e	2020-07-22	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-24	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Mitochondria

Biospecimen Locations

Blood
Brain
Cerebrospinal Fluid (CSF)
Feces
Saliva
Urine

Tissue Locations

Brain

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species
MMDBp0192540	D-3-phosphoglycerate dehydrogenase	Unknown	O43175	Homo sapiens
MMDBp0191674	D-2-hydroxyglutarate dehydrogenase, mitochondrial	Enzyme	Q8N465	Saccharomyces cerevisiae
MMDBp0191676	L-2-hydroxyglutarate dehydrogenase, mitochondrial	Enzyme	Q9H9P8	Saccharomyces cerevisiae
MMDBp0193939	Hydroxyacid-oxoacid transhydrogenase, mitochondrial	Unknown	Q8IWW8	Homo sapiens

Human Pathways

Pathways

Name	SMPDB/PathBank

Metabolic Reactions

Reaction ID	Precursor	Product	Enzyme	Reaction Type	Data Source	Reference
MMDBr0007007	Oxoglutaric acid	2-Hydroxyglutarate	Not Available	2-hydroxyglutarate dehydrogenase	VMH	30371894
MMDBr0007008	2-Hydroxyglutarate	2-Hydroxyglutaryl-CoA	Not Available	(E)-Glutaconate CoA-transferase (2-hydroxyglutarate)	VMH	30371894
MMDBr0008820	2-Hydroxyglutarate	2-Hydroxyglutarate	Solute carrier family 22 (organic anion transporter), member 8	Transport of 2-Hydroxy-Glutarate, Extracellular, Antiport	VMH	30371894
MMDBr0008821	2-Hydroxyglutarate	2-Hydroxyglutarate	Not Available	Transport of 2-Hydroxy-Glutarate, Extracellular	VMH	30371894
MMDBr0009370	Oxoglutaric acid	FAD	L-2-hydroxyglutarate dehydrogenase	2-Hydroxyglutarate: (Acceptor) 2-Oxidoreductase	VMH	30371894
MMDBr0009371	Oxoglutaric acid	2-Hydroxyglutarate	Phosphoglycerate dehydrogenase	HMR_0719	VMH	30371894
MMDBr0013218	2-Hydroxyglutarate	alpha-Ketoglutarate	Hydroxycarboxylate dehydrogenase B	Dehydrogenation; Oxidation	RHEA	34755880
MMDBr0016585	2-Hydroxyglutarate	alpha-Ketoglutarate	Hydroxycarboxylate dehydrogenase A	Dehydrogenation	RHEA	34755880
MMDBr0038841	2-Hydroxyglutaryl-CoA	2-Hydroxyglutarate	Thioesterase TesA	Hydrolysis	RHEA	34755880

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
Cerebrotendinous xanthomatosis		Association	Human Urine	HMDB	16445943
D-2-hydroxyglutaric aciduria	Increased	Association	Human Blood	HMDB	8134166
D-2-hydroxyglutaric aciduria	Increased	Association	Human Urine	HMDB	11999977
L-2-hydroxyglutaric aciduria	Increased	Association	Human Blood	HMDB	8134166
L-2-hydroxyglutaric aciduria	Increased	Association	Human Urine	HMDB	9187477
Eosinophilic esophagitis	Slight Increase	Association	Human Urine	HMDB	Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
D-2-hydroxyglutaric aciduria		Association	Human Cerebrospinal Fluid (CSF)	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
D-2-hydroxyglutaric aciduria		Association	Human Blood	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
D-2-hydroxyglutaric aciduria	Slight Decrease	Association	Human Urine	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Firmicutes	14	Human feces; Pig	Indirect association between microbiota composition and metabolites measured in host biospecimen; Metabolic reconstruction
Bacteria	Bacteroidetes	1	Human feces; Human stool	Indirect association between microbiota composition and metabolites measured in host biospecimen
Bacteria	Fusobacteria	17	Human feces; Human	Metabolic reconstruction
Bacteria	Proteobacteria	3	Human
Eukaryota/Fungi	Ascomycota	1	Human feces; Human saliva	Unknown

Exposure Sources

Source Type	Source Sub-type	Species	Data Source	Reference

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Std	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
Human Urine	Detected and Quantified	2.2		0.8	52	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	2702744
Human Urine	Detected and Quantified	11.2		4.6	33.6	umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	51.9		13.7	97.3	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	41.5		14.6	150.3	umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	12.4		5.1	24.9	umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	8087979
Human Blood	Detected and Quantified	0.7	0.2			uM	Adult (>18 years old)	Both	Normal	HMDB	8134166
Human Blood	Detected and Quantified	61	14			uM	Adult (>18 years old)	Both	D-2-Hydroxyglutaric aciduria	HMDB	8134166
Human Blood	Detected and Quantified	47	13			uM	Adult (>18 years old)	Both	L-2-hydroxyglutaric aciduria	HMDB	8134166
Human Blood	Detected and Quantified	1.5		0	1.5	uM	Adult (>18 years old)	Both	Normal	HMDB	8412012
Human Cerebrospinal fluid (csf)	Detected and Quantified	1				uM	Children (1-13 years old)	Both	Normal	HMDB	8412012
Human Cerebrospinal fluid (csf)	Detected and Quantified	1	1/0			uM	Adult (>18 years old)	Not Specified	Normal	HMDB	8412012
Human Urine	Detected and Quantified			630	1420	umol/mmol creatinine	Children (1-13 years old)	Both	L-2-hydroxyglutaric aciduria	HMDB	9187477
Human Urine	Detected and Quantified	<15				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	9187477
Human Urine	Detected and Quantified	373				umol/mmol creatinine	Children (1-13 years old)	Male	D, L-2-hydroxygluraric aciduria	HMDB	10963100
Human Urine	Detected and Quantified			685	786	umol/mmol creatinine	Infant (0-1 year old)	Male	D-2-hydroxyglutaric aciduria	HMDB	11999977
Human Urine	Detected and Quantified	4				umol/mmol creatinine	Adult (>18 years old)	Male	27-hydroxylase deficiency	HMDB	16445943
Human Urine	Detected and Quantified			4	36	umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	16445943
Human Urine	Detected and Quantified	62				umol/mmol creatinine	Newborn (0-30 days old)	Female	Deafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome	HMDB	17994565
Human Feces	Detected but not Quantified						Adult (>18 years old)	Not Specified	Normal	HMDB	20669995
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Urine	Detected and Quantified	113				umol/mmol creatinine	Newborn (0-30 days old)	Male	3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)	HMDB	22579592
Human Urine	Detected and Quantified	<5				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	22579592
Human Urine	Detected and Quantified	33		13.3	77.9	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	24029555
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	25037050
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Colorectal cancer	HMDB	25037050
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Colorectal cancer	HMDB	27015276
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Colorectal Cancer	HMDB	27275383
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27543620
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	29808030
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Supragingival Plaque	HMDB	31026179
Human Urine	Detected and Quantified			0	1.2	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Urine	Detected and Quantified			1	4	umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	31963255
Human Urine	Detected and Quantified			1	3.7	umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	31963255
Human Urine	Detected and Quantified			1.2	5.1	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Blood	Detected and Quantified	0.6		0.3	0.9	uM	Adult (>18 years old)	Both	D-2-hydroxyglutaric aciduria	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Cerebrospinal fluid (csf)	Detected and Quantified	0.2		0.1	0.3	uM	Adult (>18 years old)	Both	D-2-hydroxyglutaric aciduria	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	Zerihun T. Dame, Farid Aziat, Rupasri Mandal, Ram Krishnamurthy, Souhaila Bouatra, Shima Borzouie, An Chi Guo, Tanvir Sajed, Lu Deng, Hong Lin, Philip Liu, Edison Dong, David S. Wishart "The human saliva metabolome" Metabolomics (2015) DOI 10.1007/s11306-015-0840-5
Human Saliva	Detected and Quantified	0.881	1.13			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto, M., Saruta, J., Matsuki, C. et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles. Metabolomics, 9, 454–463 (2013). https://doi.org/10.1007/s11306-012-0464-y
Human Saliva	Detected and Quantified	1.44	1.33			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto, M., Saruta, J., Matsuki, C. et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles. Metabolomics, 9, 454–463 (2013). https://doi.org/10.1007/s11306-012-0464-y
Human Saliva	Detected and Quantified	1.08	0.629			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto, M., Saruta, J., Matsuki, C. et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles. Metabolomics, 9, 454–463 (2013). https://doi.org/10.1007/s11306-012-0464-y
Human Urine	Detected and Quantified	24.379				umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	61.074	36.982			umol/mmol creatinine	Children (1-13 years old)	Both	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	10		3	17	umol/mmol creatinine	Adult (>18 years old)	Both	D-2-Hydroxyglutaric aciduria	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	2175		50	4300	umol/mmol creatinine	Children (1-13 years old)	Both	Glutaric aciduria II	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	33		20	46	umol/mmol creatinine	Adult (>18 years old)	Both	Glutaric aciduria II	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	113				umol/mmol creatinine	Newborn (0-30 days old)	Male	3-Hydroxyacyl-CoA dehydrogenase deficiency (SCHAD)	HMDB	22579592
Human Urine	Detected and Quantified	4				umol/mmol creatinine	Adult (>18 years old)	Male	27-hydroxylase deficiency	HMDB	16445943
Human Blood	Detected and Quantified	61.0	14.0			uM	Adult (>18 years old)	Both	D-2-Hydroxyglutaric aciduria	HMDB	8134166
Human Urine	Detected and Quantified			685	786	umol/mmol creatinine	Infant (0-1 year old)	Male	D-2-hydroxyglutaric aciduria	HMDB	11999977
Human Urine	Detected and Quantified	373				umol/mmol creatinine	Children (1-13 years old)	Male	D, L-2-hydroxygluraric aciduria	HMDB	10963100
Human Urine	Detected and Quantified	62				umol/mmol creatinine	Newborn (0-30 days old)	Female	Deafness, Onychodystrophy, Osteodystrophy, Mental Retardation, and Seizures Syndrome	HMDB	17994565
Human Blood	Detected and Quantified	47.0	13.0			uM	Adult (>18 years old)	Both	L-2-hydroxyglutaric aciduria	HMDB	8134166
Human Urine	Detected and Quantified			630	1420	umol/mmol creatinine	Children (1-13 years old)	Both	L-2-hydroxyglutaric aciduria	HMDB	9187477
Human Cerebrospinal fluid (csf)	Detected and Quantified	1				uM	Children (1-13 years old)	Both	Normal	HMDB	8412012
Human Urine	Detected and Quantified	<15				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	9187477
Human Urine	Detected and Quantified	<5				umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	22579592
Human Urine	Detected and Quantified			0	1.2	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Blood	Detected and Quantified	0.7	0.2			uM	Adult (>18 years old)	Both	Normal	HMDB	8134166
Human Cerebrospinal fluid (csf)	Detected and Quantified	1.0	1/0			uM	Adult (>18 years old)	Not Specified	Normal	HMDB	8412012
Human Urine	Detected and Quantified			1.0	3.7	umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	31963255
Human Urine	Detected and Quantified			1.0	4	umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	HMDB	31963255
Human Urine	Detected and Quantified			1.2	5.1	umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	HMDB	31963255
Human Blood	Detected and Quantified	1.5		0.0	1.5	uM	Adult (>18 years old)	Both	Normal	HMDB	8412012
Human Urine	Detected and Quantified	11.2		4.6	33.6	umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	11.2		4.6	33.6	umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	12.4		5.1	24.9	umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	2.2		0.8	52	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	2702744
Human Urine	Detected and Quantified	33.0		13.3	77.9	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified			4	36	umol/mmol creatinine	Not Specified	Not Specified	Normal	HMDB	16445943
Human Urine	Detected and Quantified	41.5		14.6	150.3	umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	51.9		13.7	97.3	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	8087979
Human Urine	Detected and Quantified	61.074	36.982			umol/mmol creatinine	Children (1-13 years old)	Both	Eosinophilic esophagitis	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Cerebrospinal fluid (csf)	Detected and Quantified	0.2		0.1	0.30	uM	Adult (>18 years old)	Both	D-2-hydroxyglutaric aciduria	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Blood	Detected and Quantified	0.6		0.30	0.90	uM	Adult (>18 years old)	Both	D-2-hydroxyglutaric aciduria	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	10.0		3.0	17.0	umol/mmol creatinine	Adult (>18 years old)	Both	D-2-Hydroxyglutaric aciduria	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	2175.0		50.0	4300.0	umol/mmol creatinine	Children (1-13 years old)	Both	Glutaric aciduria II	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	33.0		20.0	46.0	umol/mmol creatinine	Adult (>18 years old)	Both	Glutaric aciduria II	HMDB	MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de)
Human Urine	Detected and Quantified	24.379				umol/mmol creatinine	Children (1-13 years old)	Not Specified	Normal	HMDB	Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work)
Human Urine	Detected and Quantified	<26.15				umol/mmol creatinine	Children (1-13 years old)	Both	Normal	HMDB	BC Children's Hospital Biochemical Genetics Lab
Human Saliva	Detected and Quantified	0.881	1.13			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	1.08	0.629			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Saliva	Detected and Quantified	1.44	1.33			uM	Adult (>18 years old)	Female	Normal	HMDB	Sugimoto et al. (2013) Physiological and environmental parameters associated with mass spectrometry-based salivary metabolomic profiles.
Human Brain	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597
Human Feces	Detected but not Quantified						Adult (>18 years old)	Not Specified	Normal	HMDB	20669995
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Saliva	Detected but not Quantified						Adult (>18 years old)	Male	Normal	HMDB	22308371
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	24029555
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	27543620
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	25037050
Human Feces	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	29808030

External Links

HMDB ID

HMDB0059655

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

Not Available

KNApSAcK ID

Not Available

Chemspider ID

KEGG Compound ID

C02630

BioCyc ID

2-HYDROXYGLUTARIC_ACID

BiGG ID

Not Available

Wikipedia Link

Alpha-Hydroxyglutaric_acid

METLIN ID

Not Available

PubChem Compound

PDB ID

Not Available

ChEBI ID

17084

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

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. doi: 10.1002/ana.410320111. [PubMed:1642474 ]
Bleiweis AS, Reeves HC, Ajl SJ: Formation of alpha-hydroxyglutaric acid by Aspergillus glaucus. J Bacteriol. 1967 Nov;94(5):1560-4. doi: 10.1128/jb.94.5.1560-1564.1967. [PubMed:6057807 ]
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 ]
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. doi: 10.1203/00006450-199309000-00007. [PubMed:8134166 ]
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. doi: 10.1055/s-2007-973722. [PubMed:9453028 ]
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. doi: 10.1002/1531-8249(199901)45:1<111::aid-art17>3.0.co;2-n. [PubMed:9894884 ]
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. doi: 10.1007/s004150050367. [PubMed:10399870 ]
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. doi: 10.1002/1099-0801(200008)14:5<317::AID-BMC989>3.0.CO;2-V. [PubMed:10960831 ]
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. doi: 10.1002/ajmg.a.20120. [PubMed:12884432 ]
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. doi: 10.1046/j.1365-2362.2003.01237.x. [PubMed:14511354 ]

Showing metabocard for 2-Hydroxyglutarate (MMDBc0000675)

MOL for #<Metabolite:0x000055d24774ca38>

3D MOL for #<Metabolite:0x000055d24774ca38>

3D SDF for #<Metabolite:0x000055d24774ca38>

3D-SDF for #<Metabolite:0x000055d24774ca38>

PDB for #<Metabolite:0x000055d24774ca38>

3D PDB for #<Metabolite:0x000055d24774ca38>

SMILES for #<Metabolite:0x000055d24774ca38>

INCHI for #<Metabolite:0x000055d24774ca38>

Structure for #<Metabolite:0x000055d24774ca38>

3D Structure for #<Metabolite:0x000055d24774ca38>