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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 20:56:00 UTC

HMDB ID

HMDB0000337

Secondary Accession Numbers

HMDB0006118
HMDB00337
HMDB06118

Metabolite Identification

Common Name

(S)-3,4-Dihydroxybutyric acid

Description

3,4-Dihydroxybutyric acid (also known as 3,4-Dihydroxybutanoic acid or 3,4-DB, or 2-deoxytetronic acid, or 2-DTA) is a normal human urinary metabolite that is excreted in increased concentration in patients with succinic semialdehyde dehydrogenase (SSADH) deficiency (PMID 12127325 ), which is a genetic disorder. SSADH deficiency is caused by an enzyme deficiency in GABA degradation. The most constant features with this disease are developmental delay, hypotonia and intellectual disability. Nearly half of SSADH patients exhibit ataxia, behavioral problems, seizures, and hyporeflexia. Normal adults excrete 0.37 +/- 0.15 (SD) mmoles of 3,4-dihydroxybutyrate per 24 hr. 3,4-dihydroxybutyrate is also detectable in blood. The renal clearance of 3,4-dihydroxybutyrate is roughly equal that of creatinine. 3,4-dihydroxybutyric acid has also been found in foods and is believed to be formed via the degradation (cooking) of di- and poly-saccharides (PMID: 1167165 ). 3,4-Dihydroxybutyric acid is believed to play a role in satiety or the feeling of fullness and suppresses food intake (PMID: 3191387 ; PMID: 6720928 ). 3,4-dihydroxybutyric acid is also elevated in the serum of individuals with dementia and may even serve as a predictive biomarker for dementia (PMID: 25177334 ). 3,4-Dihydroxybutyric acid is also a metabolite produced in Escherichia (PMID: 28342964 ).

Structure

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

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
(S)-3,4-Dihydroxybutyrate	Generator
(S)-3,4-Dihydroxy-butyric acid	HMDB
(S)-3,4-Dihydroxybutanoate	HMDB
(S)-3,4-Dihydroxybutanoic acid	HMDB
2-Deoxytetronate	HMDB
2-Deoxytetronic acid	HMDB
3,4-Dihydroxybutanoate	HMDB
3,4-Dihydroxybutanoic acid	HMDB
3,4-Dihydroxybutanoic acid, (S)-isomer	HMDB
3,4-Dihydroxybutanoic acid, monosodium salt	HMDB

Chemical Formula

C₄H₈O₄

Average Molecular Weight

120.1039

Monoisotopic Molecular Weight

120.042258744

IUPAC Name

3,4-dihydroxybutanoic acid

Traditional Name

3,4-dihydroxybutanoic acid

CAS Registry Number

51267-44-8

SMILES

OCC(O)CC(O)=O

InChI Identifier

InChI=1S/C4H8O4/c5-2-3(6)1-4(7)8/h3,5-6H,1-2H2,(H,7,8)

InChI Key

DZAIOXUZHHTJKN-UHFFFAOYSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as beta hydroxy acids and derivatives. Beta hydroxy acids and derivatives are compounds containing a carboxylic acid substituted with a hydroxyl group on the C3 carbon atom.

Kingdom

Organic compounds

Super Class

Organic acids and derivatives

Class

Hydroxy acids and derivatives

Sub Class

Beta hydroxy acids and derivatives

Direct Parent

Beta hydroxy acids and derivatives

Alternative Parents

Substituents

Beta-hydroxy acid
Short-chain hydroxy acid
Fatty acid
1,2-diol
Secondary alcohol
Carboxylic acid derivative
Carboxylic acid
Monocarboxylic acid or derivatives
Organic oxygen compound
Organooxygen compound
Primary alcohol
Hydrocarbon derivative
Carbonyl group
Organic oxide
Alcohol
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

omega-hydroxy fatty acid (CHEBI:86371 )

Ontology

Physiological effect

Not Available

Disposition

Biological location

Non-excretory biofluid

Biofluid or Excreta

Blood (PMID: 8412012)
Cerebrospinal Fluid (CSF) (PMID: 8412012)
Cerebrospinal fluid (HMDB: HMDB0000337)

Excreta

Biofluid or Excreta

Urine (PMID: 1167165)
Feces (PMID: 20669995)

Source

Endogenous

Endogenous (HMDB: HMDB0000337)

Microbe

Escherichia coli (HMDB: HMDB0000337)

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)

Process

Not Available

Role

Industrial application

Pharmaceutical industry

Biomarker

Aldehyde dehydrogenase deficiency (MarkerDB: MDB00000147)

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	Not Available	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	Not Available	Not Available
LogP	Not Available	Not Available

Experimental Chromatographic Properties

Not Available

Predicted Molecular Properties

Property	Value	Source
Water Solubility	832 g/L	ALOGPS
logP	-1.5	ALOGPS
logP	-1.4	ChemAxon
logS	0.84	ALOGPS
pKa (Strongest Acidic)	4.09	ChemAxon
pKa (Strongest Basic)	-2.9	ChemAxon
Physiological Charge	-1	ChemAxon
Hydrogen Acceptor Count	4	ChemAxon
Hydrogen Donor Count	3	ChemAxon
Polar Surface Area	77.76 Å²	ChemAxon
Rotatable Bond Count	3	ChemAxon
Refractivity	25.01 m³·mol⁻¹	ChemAxon
Polarizability	10.91 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
DarkChem	[M+H]+	126.096	31661259
DarkChem	[M-H]-	118.655	31661259
AllCCS	[M+H]+	129.482	32859911
AllCCS	[M-H]-	123.563	32859911
DeepCCS	[M+H]+	133.012	30932474
DeepCCS	[M-H]-	130.249	30932474
DeepCCS	[M-2H]-	166.603	30932474
DeepCCS	[M+Na]+	141.493	30932474
AllCCS	[M+H]+	129.5	32859911
AllCCS	[M+H-H2O]+	125.3	32859911
AllCCS	[M+NH4]+	133.4	32859911
AllCCS	[M+Na]+	134.5	32859911
AllCCS	[M-H]-	123.6	32859911
AllCCS	[M+Na-2H]-	126.8	32859911
AllCCS	[M+HCOO]-	130.3	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
(S)-3,4-Dihydroxybutyric acid	OCC(O)CC(O)=O	2636.1	Standard polar	33892256
(S)-3,4-Dihydroxybutyric acid	OCC(O)CC(O)=O	1148.1	Standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid	OCC(O)CC(O)=O	1210.7	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
(S)-3,4-Dihydroxybutyric acid,1TMS,isomer #1	C[Si](C)(C)OCC(O)CC(=O)O	1307.9	Semi standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid,1TMS,isomer #2	C[Si](C)(C)OC(CO)CC(=O)O	1282.7	Semi standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid,1TMS,isomer #3	C[Si](C)(C)OC(=O)CC(O)CO	1269.9	Semi standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid,2TMS,isomer #1	C[Si](C)(C)OCC(CC(=O)O)O[Si](C)(C)C	1379.9	Semi standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid,2TMS,isomer #2	C[Si](C)(C)OCC(O)CC(=O)O[Si](C)(C)C	1366.6	Semi standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid,2TMS,isomer #3	C[Si](C)(C)OC(=O)CC(CO)O[Si](C)(C)C	1339.2	Semi standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid,3TMS,isomer #1	C[Si](C)(C)OCC(CC(=O)O[Si](C)(C)C)O[Si](C)(C)C	1432.9	Semi standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid,1TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OCC(O)CC(=O)O	1561.3	Semi standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid,1TBDMS,isomer #2	CC(C)(C)[Si](C)(C)OC(CO)CC(=O)O	1539.4	Semi standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid,1TBDMS,isomer #3	CC(C)(C)[Si](C)(C)OC(=O)CC(O)CO	1496.4	Semi standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid,2TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OCC(CC(=O)O)O[Si](C)(C)C(C)(C)C	1842.2	Semi standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid,2TBDMS,isomer #2	CC(C)(C)[Si](C)(C)OCC(O)CC(=O)O[Si](C)(C)C(C)(C)C	1799.7	Semi standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid,2TBDMS,isomer #3	CC(C)(C)[Si](C)(C)OC(=O)CC(CO)O[Si](C)(C)C(C)(C)C	1776.9	Semi standard non polar	33892256
(S)-3,4-Dihydroxybutyric acid,3TBDMS,isomer #1	CC(C)(C)[Si](C)(C)OCC(CC(=O)O[Si](C)(C)C(C)(C)C)O[Si](C)(C)C(C)(C)C	2061.3	Semi standard non polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental GC-MS	GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-EI-TOF (Non-derivatized)	splash10-0012-0910000000-4054231c9c4cffa8a3c9	2017-09-12	HMDB team, MONA, MassBank	View Spectrum
Experimental GC-MS	GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-EI-TOF (Non-derivatized)	splash10-0012-0910000000-4054231c9c4cffa8a3c9	2018-05-18	HMDB team, MONA, MassBank	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (Non-derivatized) - 70eV, Positive	splash10-08iu-9000000000-92e0a2008c94b2db1bc7	2017-09-01	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (3 TMS) - 70eV, Positive	splash10-00di-9282000000-4da1d5189d3237b2686b	2017-10-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (TMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (TMS_1_3) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (TMS_2_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (TMS_2_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (TMS_2_3) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (TBDMS_1_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (TBDMS_1_3) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (TBDMS_2_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (TBDMS_2_2) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (TBDMS_2_3) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - (S)-3,4-Dihydroxybutyric acid GC-MS (TBDMS_3_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-3,4-Dihydroxybutyric acid 10V, Positive-QTOF	splash10-0udr-5900000000-87e855dfa4a5229ec106	2015-04-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-3,4-Dihydroxybutyric acid 20V, Positive-QTOF	splash10-0gw0-9300000000-88020d54d9b37645b873	2015-04-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-3,4-Dihydroxybutyric acid 40V, Positive-QTOF	splash10-0a4u-9000000000-d0c64e75e2e9de34f683	2015-04-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-3,4-Dihydroxybutyric acid 10V, Negative-QTOF	splash10-014i-7900000000-6aec80cde1bf2e0bca19	2015-04-25	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-3,4-Dihydroxybutyric acid 20V, Negative-QTOF	splash10-0pxr-9300000000-93d09801aa0c902a4e8f	2015-04-25	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-3,4-Dihydroxybutyric acid 40V, Negative-QTOF	splash10-0a4l-9000000000-d765ea0631d195240881	2015-04-25	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-3,4-Dihydroxybutyric acid 10V, Negative-QTOF	splash10-100r-9800000000-ffa16223efb911c9d73f	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-3,4-Dihydroxybutyric acid 20V, Negative-QTOF	splash10-052f-9000000000-bc157a746d15e9e7e4db	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-3,4-Dihydroxybutyric acid 40V, Negative-QTOF	splash10-052f-9000000000-7757ca7f030b13718ba5	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-3,4-Dihydroxybutyric acid 10V, Positive-QTOF	splash10-0zg3-9300000000-c6d15c164679f4cee318	2021-09-23	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-3,4-Dihydroxybutyric acid 20V, Positive-QTOF	splash10-0a4u-9000000000-2bf7e4eaae1145abfaf6	2021-09-23	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - (S)-3,4-Dihydroxybutyric acid 40V, Positive-QTOF	splash10-0006-9000000000-c0f696c0c09a7059f8aa	2021-09-23	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	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

Not Available

Biospecimen Locations

Blood
Cerebrospinal Fluid (CSF)
Feces
Urine

Tissue Locations

Not Available

Pathways

Not Available

Name	SMPDB/PathBank	KEGG

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	18.0 (0.0-54.0) uM	Adult (>18 years old)	Both	Normal	8412012	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	15.0 +/- 15.0 uM	Adult (>18 years old)	Both	Normal	8412012	details
Feces	Detected but not Quantified	Not Quantified	Not Specified	Not Specified	Normal	20669995	details
Feces	Detected but not Quantified	Not Quantified	Infant (0-1 year old)	Both	Normal	22411374	details
Urine	Detected and Quantified	67.400 +/- 56.200 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	12127325	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	21.2 (9.3-47.0) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	8087979	details
Urine	Detected and Quantified	23.9 (12.4-31.8) umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	8087979	details
Urine	Detected and Quantified	10.2 (6.4-16.7) umol/mmol creatinine	Children (1-13 years old)	Both	Normal	8087979	details
Urine	Detected and Quantified	8.3 (4.8-22.4) umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	8087979	details
Urine	Detected and Quantified	73.0 +/- 24.0 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Geigy Scientific ...	details
Urine	Detected and Quantified	8.0 +/- 3.0 umol/mmol creatinine	Adult (>18 years old)	Both	Normal	Geigy Scientific ...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	28157703	details
Urine	Detected and Quantified	34.9 (12.5 - 57.2) umol/mmol creatinine	Adult (>18 years old)	Not Specified	Normal	1167165	details
Urine	Detected and Quantified	42.2 (21.9-56.1) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	5.26 (4.24-6.80) 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
Urine	Detected and Quantified	137.5 (50.0-225.0) umol/mmol creatinine	Children (1-13 years old)	Both	4-Hydroxybutyric Aciduria	MetaGene: Metabol...	details
Urine	Detected and Quantified	810-1160 umol/mmol creatinine	Infant (0-1 year old)	Male	Succinic semialdehyde dehydrogenase deficiency	12127325	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Bladder cancer	28157703	details

Associated Disorders and Diseases

Disease References

Succinic semialdehyde dehydrogenase deficiency

Shinka T, Inoue Y, Ohse M, Ito A, Ohfu M, Hirose S, Kuhara T: Rapid and sensitive detection of urinary 4-hydroxybutyric acid and its related compounds by gas chromatography-mass spectrometry in a patient with succinic semialdehyde dehydrogenase deficiency. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Aug 25;776(1):57-63. [PubMed:12127325 ]
G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.

Associated OMIM IDs

271980 (Succinic semialdehyde dehydrogenase deficiency)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB021961

KNApSAcK ID

C00052139

Chemspider ID

133026

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

Not Available

PubChem Compound

150929

PDB ID

Not Available

ChEBI ID

86371

Food Biomarker Ontology

Not Available

VMH ID

Not Available

MarkerDB ID

MDB00000147

Good Scents ID

Not Available

References

Synthesis Reference

Cho, Yik-haeng; Chun, Jongpil; Park, Youngmi; Roh, Kyoungrok; Yu, Hosung; Hwang, Daeil. Process for preparing optically pure (S)-3,4-dihydroxybutyric acid derivatives. PCT Int. Appl. (2000), 29 pp.

Material Safety Data Sheet (MSDS)

Not Available

General References

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 ]
Ohdoi C, Nyhan WL, Kuhara T: Chemical diagnosis of Lesch-Nyhan syndrome using gas chromatography-mass spectrometry detection. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 15;792(1):123-30. [PubMed:12829005 ]
Nakajima MO, Etoh Y, Yanai M, Kawamura R, Kaneko K, Aoyagi K, Koiso K: [Novel renal function marker, ATP--establishing the normal range, cases of anti-tumor drags administration for urinary-track tumor, diabetic diseases and a newborn baby]. Rinsho Byori. 2002 May;50(5):513-8. [PubMed:12078051 ]
Fell V, Lee CR, Pollitt RJ: The occurrence of (S)-3,4-dihydroxybutyrate in human blood and urine. Biochem Med. 1975 May;13(1):40-5. [PubMed:1167165 ]
Shinka T, Inoue Y, Ohse M, Ito A, Ohfu M, Hirose S, Kuhara T: Rapid and sensitive detection of urinary 4-hydroxybutyric acid and its related compounds by gas chromatography-mass spectrometry in a patient with succinic semialdehyde dehydrogenase deficiency. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Aug 25;776(1):57-63. [PubMed:12127325 ]
Minami T, Oomura Y, Nabekura J, Fukuda A: Direct effects of 3,4-dihydroxybutanoic acid gamma-lactone and 2,4,5-trihydroxypentanoic acid gamma-lactone on lateral and ventromedial hypothalamic neurons. Brain Res. 1988 Oct 18;462(2):258-64. [PubMed:3191387 ]
Shimizu N, Oomura Y, Sakata T: Modulation of feeding by endogenous sugar acids acting as hunger or satiety factors. Am J Physiol. 1984 Apr;246(4 Pt 2):R542-50. doi: 10.1152/ajpregu.1984.246.4.R542. [PubMed:6720928 ]
Mousavi M, Jonsson P, Antti H, Adolfsson R, Nordin A, Bergdahl J, Eriksson K, Moritz T, Nilsson LG, Nyberg L: Serum metabolomic biomarkers of dementia. Dement Geriatr Cogn Dis Extra. 2014 Jul 11;4(2):252-62. doi: 10.1159/000364816. eCollection 2014 May. [PubMed:25177334 ]
Wang J, Shen X, Jain R, Wang J, Yuan Q, Yan Y: Establishing a novel biosynthetic pathway for the production of 3,4-dihydroxybutyric acid from xylose in Escherichia coli. Metab Eng. 2017 May;41:39-45. doi: 10.1016/j.ymben.2017.03.003. Epub 2017 Mar 23. [PubMed:28342964 ]

Showing metabocard for (S)-3,4-Dihydroxybutyric acid (HMDB0000337)

MOL for HMDB0000337 ((S)-3,4-Dihydroxybutyric acid)

3D MOL for HMDB0000337 ((S)-3,4-Dihydroxybutyric acid)

3D SDF for HMDB0000337 ((S)-3,4-Dihydroxybutyric acid)

3D-SDF for HMDB0000337 ((S)-3,4-Dihydroxybutyric acid)

PDB for HMDB0000337 ((S)-3,4-Dihydroxybutyric acid)

3D PDB for HMDB0000337 ((S)-3,4-Dihydroxybutyric acid)

SMILES for HMDB0000337 ((S)-3,4-Dihydroxybutyric acid)

INCHI for HMDB0000337 ((S)-3,4-Dihydroxybutyric acid)

Structure for HMDB0000337 ((S)-3,4-Dihydroxybutyric acid)

3D Structure for HMDB0000337 ((S)-3,4-Dihydroxybutyric acid)

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra

IR Spectra