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Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2023-05-30 20:55:57 UTC

HMDB ID

HMDB0000008

Secondary Accession Numbers

HMDB00008

Metabolite Identification

Common Name

2-Hydroxybutyric acid

Description

2-Hydroxybutyric acid (CAS: 600-15-7), also known as alpha-hydroxybutyrate, is an organic acid derived from alpha-ketobutyrate. alpha-Ketobutyrate is produced by amino acid catabolism (threonine and methionine) and glutathione anabolism (cysteine formation pathway) and is metabolized into propionyl-CoA and carbon dioxide (PMID: 20526369 ). 2-Hydroxybutyric acid is formed as a byproduct from the formation of alpha-ketobutyrate via a reaction catalyzed by lactate dehydrogenase (LDH) or alpha-hydroxybutyrate dehydrogenase (alphaHBDH). alpha-Hydroxybutyric acid is primarily produced in mammalian hepatic tissues that catabolize L-threonine or synthesize glutathione. Oxidative stress or detoxification of xenobiotics in the liver can dramatically increase the rate of hepatic glutathione synthesis. Under such metabolic stress conditions, supplies of L-cysteine for glutathione synthesis become limiting, so homocysteine is diverted from the transmethylation pathway (which forms methionine) into the transsulfuration pathway (which forms cystathionine). alpha-Ketobutyrate is released as a byproduct when cystathionine is cleaved into cysteine that is incorporated into glutathione. Chronic shifts in the rate of glutathione synthesis may be reflected by urinary excretion of 2-hydroxybutyrate. 2-Hydroxybutyrate is an early marker for both insulin resistance and impaired glucose regulation that appears to arise due to increased lipid oxidation and oxidative stress (PMID: 20526369 ). 2-Hydroxybutyric acid is often found in the urine of patients suffering from lactic acidosis and ketoacidosis. 2-Hydroxybutyric acid generally appears at high concentrations in situations related to deficient energy metabolism (e.g. birth asphyxia) and also in inherited metabolic diseases affecting the central nervous system during neonatal development, such as "cerebral" lactic acidosis, glutaric aciduria type II, dihydrolipoyl dehydrogenase (E3) deficiency, and propionic acidemia. More recently it has been noted that elevated levels of alpha-hydroxybutyrate in the plasma is a good marker for early-stage type II diabetes (PMID: 19166731 ). It was concluded from studies done in the mid-1970s that an increased NADH2/NAD ratio was the most important factor for the production of 2-hydroxybutyric acid (PMID: 168632 ).

Structure

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

  Mrv1652309142106383D          

 15 14  0  0  0  0            999 V2000
   -2.0817    0.1757    0.1709 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8778   -0.0846   -0.6959 C   0  0  2  0  0  0  0  0  0  0  0  0
    0.3603   -0.2117    0.1873 C   0  0  2  0  0  0  0  0  0  0  0  0
    0.5730    0.9558    0.9144 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.5707   -0.4592   -0.6320 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.5903   -1.4832   -1.3789 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.6722    0.3608   -0.6257 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8322    0.4420    1.2074 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7329   -0.7417    0.2381 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7371    0.9886   -0.2372 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6923    0.7513   -1.4135 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0154   -1.0471   -1.2230 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.2243   -1.0352    0.9148 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.4733    1.3514    0.7314 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.5053    0.0372   -0.1599 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  2  3  1  0  0  0  0
  3  4  1  0  0  0  0
  3  5  1  0  0  0  0
  5  6  2  0  0  0  0
  5  7  1  0  0  0  0
  1  8  1  0  0  0  0
  1  9  1  0  0  0  0
  1 10  1  0  0  0  0
  2 11  1  0  0  0  0
  2 12  1  0  0  0  0
  3 13  1  1  0  0  0
  4 14  1  0  0  0  0
  7 15  1  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000008

> <DATABASE_NAME>
hmdb

> <SMILES>
[H]OC(=O)[C@@]([H])(O[H])C([H])([H])C([H])([H])[H]

> <INCHI_IDENTIFIER>
InChI=1S/C4H8O3/c1-2-3(5)4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m0/s1

> <INCHI_KEY>
AFENDNXGAFYKQO-VKHMYHEASA-N

> <FORMULA>
C4H8O3

> <MOLECULAR_WEIGHT>
104.105

> <EXACT_MASS>
104.047344118

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
15

> <JCHEM_AVERAGE_POLARIZABILITY>
9.984831454501116

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
2

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
(2S)-2-hydroxybutanoic acid

> <ALOGPS_LOGP>
-0.16

> <JCHEM_LOGP>
0.050692876999999914

> <ALOGPS_LOGS>
0.67

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA>
14.293154365104638

> <JCHEM_PKA_STRONGEST_ACIDIC>
3.989998858113875

> <JCHEM_PKA_STRONGEST_BASIC>
-3.7935876935907205

> <JCHEM_POLAR_SURFACE_AREA>
57.53

> <JCHEM_REFRACTIVITY>
23.3638

> <JCHEM_ROTATABLE_BOND_COUNT>
2

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
4.84e+02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
(S)-2-hydroxybutyric acid

> <JCHEM_VEBER_RULE>
0

$$$$

HEADER    PROTEIN                                 14-SEP-21   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   14-SEP-21         0                                  
HETATM    1  C   UNK     0      -2.082   0.176   0.171  0.00  0.00           C+0
HETATM    2  C   UNK     0      -0.878  -0.085  -0.696  0.00  0.00           C+0
HETATM    3  C   UNK     0       0.360  -0.212   0.187  0.00  0.00           C+0
HETATM    4  O   UNK     0       0.573   0.956   0.914  0.00  0.00           O+0
HETATM    5  C   UNK     0       1.571  -0.459  -0.632  0.00  0.00           C+0
HETATM    6  O   UNK     0       1.590  -1.483  -1.379  0.00  0.00           O+0
HETATM    7  O   UNK     0       2.672   0.361  -0.626  0.00  0.00           O+0
HETATM    8  H   UNK     0      -1.832   0.442   1.207  0.00  0.00           H+0
HETATM    9  H   UNK     0      -2.733  -0.742   0.238  0.00  0.00           H+0
HETATM   10  H   UNK     0      -2.737   0.989  -0.237  0.00  0.00           H+0
HETATM   11  H   UNK     0      -0.692   0.751  -1.414  0.00  0.00           H+0
HETATM   12  H   UNK     0      -1.015  -1.047  -1.223  0.00  0.00           H+0
HETATM   13  H   UNK     0       0.224  -1.035   0.915  0.00  0.00           H+0
HETATM   14  H   UNK     0       1.473   1.351   0.731  0.00  0.00           H+0
HETATM   15  H   UNK     0       3.505   0.037  -0.160  0.00  0.00           H+0
CONECT    1    2    8    9   10                                             
CONECT    2    1    3   11   12                                             
CONECT    3    2    4    5   13                                             
CONECT    4    3   14                                                       
CONECT    5    3    6    7                                                  
CONECT    6    5                                                            
CONECT    7    5   15                                                       
CONECT    8    1                                                            
CONECT    9    1                                                            
CONECT   10    1                                                            
CONECT   11    2                                                            
CONECT   12    2                                                            
CONECT   13    3                                                            
CONECT   14    4                                                            
CONECT   15    7                                                            
MASTER        0    0    0    0    0    0    0    0   15    0   28    0
END

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
(S)-2-Hydroxybutanoic acid	ChEBI
2-Hydroxybutyrate	ChEBI
2-Hydroxybutyric acid	ChEBI
L-2-Hydroxybutanoic acid	ChEBI
L-2-Hydroxybutyric acid	ChEBI
L-alpha-Hydroxybutanoic acid	ChEBI
L-alpha-Hydroxybutyric acid	ChEBI
(S)-2-Hydroxybutanoate	Generator
L-2-Hydroxybutanoate	Generator
L-2-Hydroxybutyrate	Generator
L-a-Hydroxybutanoate	Generator
L-a-Hydroxybutanoic acid	Generator
L-alpha-Hydroxybutanoate	Generator
L-Α-hydroxybutanoate	Generator
L-Α-hydroxybutanoic acid	Generator
L-a-Hydroxybutyrate	Generator
L-a-Hydroxybutyric acid	Generator
L-alpha-Hydroxybutyrate	Generator
L-Α-hydroxybutyrate	Generator
L-Α-hydroxybutyric acid	Generator
(S)-2-Hydroxybutyrate	Generator
2-Hydroxybutanoate	Generator
(+)-2-Hydroxy-n-butyric acid	HMDB
(+)-2-Hydroxybutanoic acid	HMDB
(+)-2-Hydroxybutyric acid	HMDB
(+)-alpha-Hydroxybutyric acid	HMDB
(+)-α-Hydroxybutyric acid	HMDB
(2S)-2-Hydroxybutanoic acid	HMDB
(S)-(+)-2-Hydroxybutanoic acid	HMDB
(S)-2-Hydroxybutyric acid	HMDB
2-Hydroxybutanoic acid	HMDB
alpha-Hydroxy-n-butyric acid	HMDB
alpha-Hydroxybutanoic acid	HMDB
alpha-Hydroxybutyric acid	HMDB
α-Hydroxy-n-butyric acid	HMDB
α-Hydroxybutanoic acid	HMDB
α-Hydroxybutyric acid	HMDB

Chemical Formula

C₄H₈O₃

Average Molecular Weight

104.105

Monoisotopic Molecular Weight

104.047344118

IUPAC Name

(2S)-2-hydroxybutanoic acid

Traditional Name

(S)-2-hydroxybutyric acid

CAS Registry Number

3347-90-8

SMILES

[H]OC(=O)[C@@]([H])(O[H])C([H])([H])C([H])([H])[H]

InChI Identifier

InChI=1S/C4H8O3/c1-2-3(5)4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m0/s1

InChI Key

AFENDNXGAFYKQO-VKHMYHEASA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as alpha hydroxy acids and derivatives. These are organic compounds containing a carboxylic acid substituted with a hydroxyl group on the adjacent carbon.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Hydroxy acids and derivatives

Sub Class

Alpha hydroxy acids and derivatives

Direct Parent

Alpha hydroxy acids and derivatives

Alternative Parents

Substituents

Fatty acid
Alpha-hydroxy acid
Secondary alcohol
Monocarboxylic acid or derivatives
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-hydroxybutyric acid (CHEBI:50613 )
Hydroxy fatty acids (C05984 )
Hydroxy fatty acids (LMFA01050342 )

Ontology

Physiological effect

Health effect

Metabolic disorder

Pyruvate Dehydrogenase Deficiency (HMDB: HMDB0000008)

Nervous system disorder

Central nervous system disorder

Psychiatric disorder

Schizophrenia (HMDB: HMDB0000008)

Disposition

Biological location

Non-excretory biofluid

Biofluid or Excreta

Blood (PMID: 8412012)
Cerebrospinal Fluid (CSF) (PMID: 8376520)
Saliva (PMID: 22308371)
Cerebrospinal fluid (HMDB: HMDB0000008)

Organ

Prostate (HMDB: HMDB0000008)
Placenta (HMDB: HMDB0000008)

Excreta

Biofluid or Excreta

Urine (PMID: 168632)
Feces (PMID: 27275383)
Sweat (PMID: 26755145)

Cellular substructure

Cytoplasm (HMDB: HMDB0000008)
Extracellular (HMDB: HMDB0000008)

Source

Endogenous

Endogenous (HMDB: HMDB0000008)

Pheasant (FooDB: FOOD00430)
Squab (FooDB: FOOD00481)
Turkey (FooDB: FOOD00494)
Quail (FooDB: FOOD00541)
Anatidae (FooDB: FOOD00576)
Rock ptarmigan (FooDB: FOOD00597)
Columbidae (Dove, Pigeon) (FooDB: FOOD00610)
Chicken (FooDB: FOOD00329)
Mallard duck (FooDB: FOOD00353)
Emu (FooDB: FOOD00360)
Greylag goose (FooDB: FOOD00368)
Guinea hen (FooDB: FOOD00373)
Ostrich (FooDB: FOOD00422)
Velvet duck (FooDB: FOOD00427)

Lagomorph

Ovis

Sheep (Mutton, Lamb) (FooDB: FOOD00473)

Bovine

Venison

Deer (FooDB: FOOD00524)
Mule deer (FooDB: FOOD00348)
Elk (FooDB: FOOD00359)

Caprae

Domestic goat (FooDB: FOOD00529)

Swine

Domestic pig (FooDB: FOOD00536)
Wild boar (FooDB: FOOD00307)

Equine

Horse (FooDB: FOOD00378)

Milk and milk product

Unfermented milk

Milk (Cow) (FooDB: FOOD00618)

Process

Naturally occurring process

Biological process

Biochemical pathway

Disease pathway

Biotinidase Deficiency (HMDB: HMDB0000008)
Methylmalonic Aciduria Due to Cobalamin-Related Disorders (PathBank: SMP0120748)
Malonic Aciduria (PathBank: SMP0120734)
Malonyl-CoA Decarboxylase Deficiency (PathBank: SMP0120786)

Metabolic pathway

Propanoate Metabolism (PathBank: SMP0087480)
Propanoate Metabolism (HMDB: HMDB0000008)

Role

Biological role

Industrial application

Pharmaceutical industry

Biomarker

Preeclampsia/Eclampsia (MarkerDB: MDB00000003)
Pregnancy (MarkerDB: MDB00000003)
Pyruvate Dehydrogenase Deficiency (MarkerDB: MDB00000003)

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	44.2 °C	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	Not Available	Not Available
LogP	Not Available	Not Available

Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct Type	Data Source	CCS Value (Å²)	Reference
[M-H]-	Baker	124.667	30932474
[M-H]-	Not Available	124.667	http://allccs.zhulab.cn/database/detail?ID=AllCCS00001760

Predicted Molecular Properties

Property	Value	Source
Water Solubility	484 g/L	ALOGPS
logP	-0.16	ALOGPS
logP	0.051	ChemAxon
logS	0.67	ALOGPS
pKa (Strongest Acidic)	3.99	ChemAxon
pKa (Strongest Basic)	-3.8	ChemAxon
Physiological Charge	-1	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	2	ChemAxon
Polar Surface Area	57.53 Å²	ChemAxon
Rotatable Bond Count	2	ChemAxon
Refractivity	23.36 m³·mol⁻¹	ChemAxon
Polarizability	9.98 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Predicted Chromatographic Properties

Predicted Collision Cross Sections

Predictor	Adduct Type	CCS Value (Å²)	Reference
DeepCCS	[M+H]+	120.605	30932474
DeepCCS	[M-H]-	117.804	30932474
DeepCCS	[M-2H]-	154.261	30932474
DeepCCS	[M+Na]+	128.945	30932474
AllCCS	[M+H]+	127.7	32859911
AllCCS	[M+H-H2O]+	123.4	32859911
AllCCS	[M+NH4]+	131.8	32859911
AllCCS	[M+Na]+	132.9	32859911
AllCCS	[M-H]-	123.6	32859911
AllCCS	[M+Na-2H]-	127.4	32859911
AllCCS	[M+HCOO]-	131.7	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
2-Hydroxybutyric acid	CC[C@H](O)C(O)=O	1796.3	Standard polar	33892256
2-Hydroxybutyric acid	CC[C@H](O)C(O)=O	928.5	Standard non polar	33892256
2-Hydroxybutyric acid	CC[C@H](O)C(O)=O	990.5	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
2-Hydroxybutyric acid,1TMS,isomer #1	CC[C@H](O[Si](C)(C)C)C(=O)O	1087.2	Semi standard non polar	33892256
2-Hydroxybutyric acid,1TMS,isomer #2	CC[C@H](O)C(=O)O[Si](C)(C)C	983.5	Semi standard non polar	33892256
2-Hydroxybutyric acid,2TMS,isomer #1	CC[C@H](O[Si](C)(C)C)C(=O)O[Si](C)(C)C	1135.8	Semi standard non polar	33892256
2-Hydroxybutyric acid,1TBDMS,isomer #1	CC[C@H](O[Si](C)(C)C(C)(C)C)C(=O)O	1313.5	Semi standard non polar	33892256
2-Hydroxybutyric acid,1TBDMS,isomer #2	CC[C@H](O)C(=O)O[Si](C)(C)C(C)(C)C	1207.3	Semi standard non polar	33892256
2-Hydroxybutyric acid,2TBDMS,isomer #1	CC[C@H](O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C	1575.5	Semi standard non polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental GC-MS	GC-MS Spectrum - 2-Hydroxybutyric acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)	splash10-001j-0900000000-44dd1bf8072e5731bac4	2020-02-05	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - 2-Hydroxybutyric acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (2 TMS)	splash10-00e9-9700000000-93eba027d5343e3d6ce1	2020-02-05	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (2 TMS)	splash10-001i-1910000000-3bc9898b26df33cad244	2020-02-05	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - 2-Hydroxybutyric acid EI-B (Non-derivatized)	splash10-0a4i-9000000000-4d935da8e593ec61fd96	2020-02-05	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - 2-Hydroxybutyric acid GC-EI-TOF (Non-derivatized)	splash10-001j-0900000000-44dd1bf8072e5731bac4	2020-02-05	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - 2-Hydroxybutyric acid GC-EI-TOF (Non-derivatized)	splash10-00e9-9700000000-93eba027d5343e3d6ce1	2020-02-05	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (Non-derivatized)	splash10-001i-1910000000-3bc9898b26df33cad244	2020-02-05	HMDB team, MONA, MassBank	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (TMS_2_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 2-Hydroxybutyric acid GC-MS (TBDMS_2_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxybutyric acid Quattro_QQQ 10V, Negative-QTOF (Annotated)	splash10-0zfr-9600000000-f688c3e6fdeb5f0270c5	2020-02-05	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxybutyric acid Quattro_QQQ 25V, Negative-QTOF (Annotated)	splash10-0a4j-9200000000-e3b371c29b9ebed3199b	2020-02-05	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxybutyric acid Quattro_QQQ 40V, Negative-QTOF (Annotated)	splash10-0zfs-9700000000-bccfada2c97e04d34417	2020-02-05	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxybutyric acid EI-B (HITACHI RMU-7M) , Positive-QTOF	splash10-0a4i-9000000000-4d935da8e593ec61fd96	2020-02-05	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxybutyric acid LC-ESI-IT , negative-QTOF	splash10-0udi-2900000000-3c7915ea71886873f024	2020-02-05	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxybutyric acid , negative-QTOF	splash10-0zfr-7900000000-d91c840306a06d4ea513	2020-02-05	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxybutyric acid 40V, Negative-QTOF	splash10-0006-9000000000-94eb4d9d668d4d53231b	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxybutyric acid 10V, Negative-QTOF	splash10-0pb9-9500000000-a90d8674aafbec41b8b2	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxybutyric acid 20V, Negative-QTOF	splash10-0a4i-9400000000-7b0a49d8800ded4cdff4	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxybutyric acid 35V, Negative-QTOF	splash10-0zfr-7900000000-52227d0dd814179fde45	2021-09-20	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - 2-Hydroxybutyric acid 35V, Negative-QTOF	splash10-0zmi-9600000000-8ea58ec01176ecce4735	2021-09-20	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 10V, Positive-QTOF	splash10-0a4r-9400000000-7bdf5cb6c5aa8c1d607d	2019-02-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 20V, Positive-QTOF	splash10-0a4r-9100000000-bf04b472392c16bd165c	2019-02-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 40V, Positive-QTOF	splash10-052f-9000000000-483980f183cd4095b2f3	2019-02-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 10V, Negative-QTOF	splash10-0udi-5900000000-9185cef9251e02ac688c	2019-02-23	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 20V, Negative-QTOF	splash10-0a4r-9200000000-ea836921fe44635e66c2	2019-02-23	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 40V, Negative-QTOF	splash10-0a4l-9000000000-cf8130e8094c63aa0e25	2019-02-23	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 10V, Positive-QTOF	splash10-052o-9000000000-d175dd460cd717e71350	2021-09-23	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 20V, Positive-QTOF	splash10-0a4l-9000000000-0244ee6a1375f83df3a2	2021-09-23	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 40V, Positive-QTOF	splash10-0006-9000000000-a4c46731c8286baabca9	2021-09-23	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 10V, Negative-QTOF	splash10-0udi-2900000000-d938708d3f7f8d712710	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 20V, Negative-QTOF	splash10-0ap0-9100000000-a0763e4b908fc98b5853	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxybutyric acid 40V, Negative-QTOF	splash10-0a4i-9000000000-9077c11ebfee5a552d35	2021-09-24	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Experimental 1D NMR	¹H NMR Spectrum (1D, 600 MHz, H₂O, experimental)	2020-02-05	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-14	Wishart Lab	View Spectrum
Experimental 2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)	2020-02-05	Wishart Lab	View Spectrum

IR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M-H]-)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+H]+)	2023-02-03	FELIX lab	View Spectrum
Predicted IR Spectrum	IR Ion Spectrum (Predicted IRIS Spectrum, Adduct: [M+Na]+)	2023-02-03	FELIX lab	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Extracellular

Biospecimen Locations

Blood
Cerebrospinal Fluid (CSF)
Feces
Saliva
Sweat
Urine

Tissue Locations

Placenta
Prostate

Pathways

Name	SMPDB/PathBank	KEGG
Propanoate metabolism	Not Available
Malonic Aciduria		Not Available
Methylmalonic Aciduria Due to Cobalamin-Related Disorders		Not Available
Malonyl-coa decarboxylase deficiency		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	8.00-80.0 uM	Adult (>18 years old)	Both	Normal	Molecular You	details
Blood	Detected and Quantified	26.9(32.92) uM	Adult (>18 years old)	Both	Normal	26010610	details
Blood	Detected and Quantified	8.00-80.0 uM	Adult (>18 years old)	Both	Normal	8412012	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	22024767	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Female	Normal	27355821	details
Blood	Detected and Quantified	54.0 (8.0-80.0) uM	Adult (>18 years old)	Both	Normal	8412012	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	40.0 +/- 24.0 uM	Adult (>18 years old)	Both	Normal	18502700	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	85.0 uM	Adult (>18 years old)	Both	Normal	18502700	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	37 +/- 21 (10 - 76) uM	Children (1-13 years old)	Both	Normal	8376520	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	35.0 +/- 24.0 uM	Adult (>18 years old)	Both	Normal	8412012	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	29808030	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	25037050	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	27543620	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected and Quantified	10.42 +/- 9.20 uM	Adult (>18 years old)	Female	Normal	23857558	details
Sweat	Detected but not Quantified	Not Quantified	Adult	Both	Normal	26755145	details
Urine	Detected and Quantified	0.31-0.69 umol/mmol creatinine	Adult (>18 years old)	Female	Normal	27012787	details
Urine	Detected and Quantified	2.445 (0-4.89) umol/mmol creatinine	Not Specified	Both	Normal	168632	details
Urine	Detected and Quantified	0-3 umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	31963255	details
Urine	Detected and Quantified	0-6.0 umol/mmol creatinine	Infant (0-1 year old)	Male	Normal	31963255	details
Urine	Detected and Quantified	0-6.1 umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	31963255	details
Urine	Detected and Quantified	0-9.3 umol/mmol creatinine	Infant (0-1 year old)	Female	Normal	31963255	details
Urine	Detected and Quantified	3.29 +/- 3.529 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details
Urine	Detected and Quantified	2.8 (1.2-6.9) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	5.0 (0.0-10.0) umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	Geigy Scientific ...	details
Urine	Detected and Quantified	0.26-0.53 umol/mmol creatinine	Adult (>18 years old)	Male	Normal	27012787	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	22024767	details
Urine	Detected and Quantified	2.53 (1.56-6.08) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Analysis of 40 NI...	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	26.96 +/- 13.11 uM	Adult (>18 years old)	Female	Pregnancy with fetuses with trisomy 18	23535240	details
Blood	Detected and Quantified	16.93 +/- 8.17 uM	Adult (>18 years old)	Female	Pregnancy	23535240	details
Blood	Detected and Quantified	21.4 (5.6) uM	Adult (>18 years old)	Female	Early preeclampsia	22494326	details
Blood	Detected and Quantified	19.5 (7.4) uM	Adult (>18 years old)	Female	Pregnancy	22494326	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25105552	details
Blood	Detected and Quantified	17.13 (7.48) uM	Adult (>18 years old)	Female	Pregnancy with fetus having congenital heart defect	24704061	details
Blood	Detected and Quantified	16.69 (6.25) uM	Adult (>18 years old)	Female	Pregnancy	24704061	details
Blood	Detected and Quantified	28.0 (14.4) uM	Adult (>18 years old)	Female	Late-onset preeclampsia	23159745	details
Blood	Detected and Quantified	4.14(10.18) uM	Adult (>18 years old)	Both	Heart failure with reduced ejection fraction	26010610	details
Blood	Detected and Quantified	21.2 (7.5) uM	Adult (>18 years old)	Female	Pregnancy	23159745	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Schizophrenia	22024767	details
Blood	Detected and Quantified	23.1 (14.1) uM	Adult (>18 years old)	Female	Down syndrome pregnancy	23313728	details
Blood	Detected and Quantified	15.0 (7.4) uM	Adult (>18 years old)	Female	Pregnancy	23313728	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	25037050	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal Cancer	27275383	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal cancer	27015276	details
Saliva	Detected and Quantified	7.26 +/- 4.47 uM	Adult (>18 years old)	Male	Alzheimer's disease	23857558	details
Saliva	Detected and Quantified	7.30 +/- 1.32 uM	Adult (>18 years old)	Male	Frontotemporal lobe dementia	23857558	details
Saliva	Detected and Quantified	8.12 +/- 5.32 uM	Adult (>18 years old)	Both	Lewy body disease	23857558	details
Urine	Detected and Quantified	>25.0 umol/mmol creatinine	Adult (>18 years old)	Both	Pyruvate dehydrogenase deficiency	OMIM 238331	details
Urine	Detected and Quantified	8.699 +/- 10.366 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Schizophrenia	22024767	details
Urine	Detected and Quantified	248.732 umol/mmol creatinine	Infant (0-1 year old)	Male	Dihydrolipoamide Dehydrogenase Deficiency	6688766	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal adenoma	27510537	details

Associated Disorders and Diseases

Disease References

Colorectal cancer
Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14. [PubMed:25105552 ] Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ] Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ] Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Schizophrenia
Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767 ]
Early preeclampsia
Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ]
Pregnancy
Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326 ] Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ] Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester Down syndrome prediction. Am J Obstet Gynecol. 2013 May;208(5):371.e1-8. doi: 10.1016/j.ajog.2012.12.035. Epub 2013 Jan 8. [PubMed:23313728 ] Bahado-Singh RO, Akolekar R, Chelliah A, Mandal R, Dong E, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester trisomy 18 detection. Am J Obstet Gynecol. 2013 Jul;209(1):65.e1-9. doi: 10.1016/j.ajog.2013.03.028. Epub 2013 Mar 25. [PubMed:23535240 ] Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061 ]
Late-onset preeclampsia
Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745 ]
Alzheimer's disease
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Frontotemporal dementia
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Lewy body disease
Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013 Sep 6. [PubMed:23857558 ]
Pyruvate dehydrogenase deficiency
(). OMIM 238331. .
Eosinophilic esophagitis
Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Dihydrolipoamide Dehydrogenase Deficiency
Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. [PubMed:6688766 ]

Associated OMIM IDs

114500 (Colorectal cancer)
181500 (Schizophrenia)
104300 (Alzheimer's disease)
600274 (Frontotemporal dementia)
312170 (Pyruvate dehydrogenase deficiency)
610247 (Eosinophilic esophagitis)
246900 (Dihydrolipoamide Dehydrogenase Deficiency)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB021867

KNApSAcK ID

Not Available

Chemspider ID

389701

KEGG Compound ID

C05984

BioCyc ID

CPD-3564

BiGG ID

Not Available

Wikipedia Link

2-Hydroxybutyric_acid

METLIN ID

Not Available

PubChem Compound

440864

PDB ID

Not Available

ChEBI ID

50613

Food Biomarker Ontology

Not Available

VMH ID

Not Available

MarkerDB ID

MDB00000003

Good Scents ID

Not Available

References

Synthesis Reference

Carlier, J. P.; Henry, C.; Lorin, V.; Rouffignat, K. Conversion of DL-threonine, D-threonine and 2-oxobutyrate into propionate and 2-hydroxybutyrate by Fusobacterium species. Letters in Applied Microbiology (1997), 25(5), 371-374.

Material Safety Data Sheet (MSDS)

Not Available

General References

Sweatman BC, Farrant RD, Holmes E, Ghauri FY, Nicholson JK, Lindon JC: 600 MHz 1H-NMR spectroscopy of human cerebrospinal fluid: effects of sample manipulation and assignment of resonances. J Pharm Biomed Anal. 1993 Aug;11(8):651-64. [PubMed:8257730 ]
Landaas S, Pettersen JE: Clinical conditions associated with urinary excretion of 2-hydroxybutyric acid. Scand J Clin Lab Invest. 1975 May;35(3):259-66. [PubMed:168632 ]
Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762. [PubMed:19212411 ]
Li X, Xu Z, Lu X, Yang X, Yin P, Kong H, Yu Y, Xu G: Comprehensive two-dimensional gas chromatography/time-of-flight mass spectrometry for metabonomics: Biomarker discovery for diabetes mellitus. Anal Chim Acta. 2009 Feb 9;633(2):257-62. doi: 10.1016/j.aca.2008.11.058. Epub 2008 Dec 3. [PubMed:19166731 ]
Gall WE, Beebe K, Lawton KA, Adam KP, Mitchell MW, Nakhle PJ, Ryals JA, Milburn MV, Nannipieri M, Camastra S, Natali A, Ferrannini E: alpha-hydroxybutyrate is an early biomarker of insulin resistance and glucose intolerance in a nondiabetic population. PLoS One. 2010 May 28;5(5):e10883. doi: 10.1371/journal.pone.0010883. [PubMed:20526369 ]
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 ]
(). Aldrich Library of FT-IR Spectra, 1st edn., 1985, 1, 548D. .
(). Mori, K. et al., Tetrahedron, 1979, 35, 1601. .
(). Levene, P.A. et al., J. Biol. Chem., 1941, 141, 391. .
(). Duvillier, E., Ann. Chim. Phys., 1879, 17, 532. .
(). Chenault, H.K. et al., J.O.C., 1987, 52, 2608. .
(). Aldrich Library of 13C and 1H FT NMR Spectra, 1992, 1, 854B. .
(). Horn, D.H.S. et al., J.C.S., 1954, 1460. .
(). Wong, C.H. et al., J.O.C., 1985, 50, 1992. .

Enzymes

Enzyme Details

1. Dihydrolipoyl dehydrogenase, mitochondrial

General function:: Involved in oxidoreductase activity
Specific function:: Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
Gene Name:: DLD
Uniprot ID:: P09622
Molecular weight:: 54176.91

References

Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. [PubMed:6688766 ]

Enzyme Details

2. Sulfotransferase family cytosolic 2B member 1

General function:: Involved in sulfotransferase activity
Specific function:: Sulfotransferase that utilizes 3'-phospho-5'-adenylyl sulfate (PAPS) as sulfonate donor to catalyze the sulfate conjugation of many hormones, neurotransmitters, drugs and xenobiotic compounds. Sulfonation increases the water solubility of most compounds, and therefore their renal excretion, but it can also result in bioactivation to form active metabolites. Sulfates hydroxysteroids like DHEA. Isoform 1 preferentially sulfonates cholesterol, and isoform 2 avidly sulfonates pregnenolone but not cholesterol.
Gene Name:: SULT2B1
Uniprot ID:: O00204
Molecular weight:: 39598.595

Reactions

2-Hydroxybutyric acid → 2-(sulfooxy)butanoic acid	details

Enzyme Details

3. UDP-glucuronosyltransferase 1-1

General function:: Involved in transferase activity, transferring hexosyl groups
Specific function:: UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isoform glucuronidates bilirubin IX-alpha to form both the IX-alpha-C8 and IX-alpha-C12 monoconjugates and diconjugate. Is also able to catalyze the glucuronidation of 17beta-estradiol, 17alpha-ethinylestradiol, 1-hydroxypyrene, 4-methylumbelliferone, 1-naphthol, paranitrophenol, scopoletin, and umbelliferone.
Gene Name:: UGT1A1
Uniprot ID:: P22309
Molecular weight:: 59590.91

Reactions

2-Hydroxybutyric acid → 6-(1-carboxypropoxy)-3,4,5-trihydroxyoxane-2-carboxylic acid	details
2-Hydroxybutyric acid → 3,4,5-trihydroxy-6-[(2-hydroxybutanoyl)oxy]oxane-2-carboxylic acid	details

Enzyme Details

4. L-lactate dehydrogenase A-like 6A

General function:: Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:: Displays an lactate dehydrogenase activity. Significantly increases the transcriptional activity of JUN, when overexpressed.
Gene Name:: LDHAL6A
Uniprot ID:: Q6ZMR3
Molecular weight:: 36507.015

Reactions

2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Ion	details

Enzyme Details

5. L-lactate dehydrogenase B chain

General function:: Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:: Not Available
Gene Name:: LDHB
Uniprot ID:: P07195
Molecular weight:: 36638.225

Reactions

2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Ion	details

Enzyme Details

6. L-lactate dehydrogenase C chain

General function:: Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:: Possible role in sperm motility.
Gene Name:: LDHC
Uniprot ID:: P07864
Molecular weight:: 36310.965

Reactions

2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Ion	details

Enzyme Details

7. L-lactate dehydrogenase A chain

General function:: Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:: Not Available
Gene Name:: LDHA
Uniprot ID:: P00338
Molecular weight:: 30204.975

Reactions

2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Ion	details

Enzyme Details

8. L-lactate dehydrogenase A-like 6B

General function:: Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:: Not Available
Gene Name:: LDHAL6B
Uniprot ID:: Q9BYZ2
Molecular weight:: 41942.53

Reactions

2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Ion	details

Showing metabocard for 2-Hydroxybutyric acid (HMDB0000008)

MOL for HMDB0000008 (2-Hydroxybutyric acid)

3D MOL for HMDB0000008 (2-Hydroxybutyric acid)

3D SDF for HMDB0000008 (2-Hydroxybutyric acid)

3D-SDF for HMDB0000008 (2-Hydroxybutyric acid)

PDB for HMDB0000008 (2-Hydroxybutyric acid)

3D PDB for HMDB0000008 (2-Hydroxybutyric acid)

SMILES for HMDB0000008 (2-Hydroxybutyric acid)

INCHI for HMDB0000008 (2-Hydroxybutyric acid)

Structure for HMDB0000008 (2-Hydroxybutyric acid)

3D Structure for HMDB0000008 (2-Hydroxybutyric acid)

Experimental Collision Cross Sections

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra

IR Spectra

Enzymes

References

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