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Showing metabocard for 3-Hydroxybutyric acid (HMDB0000011)

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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-05-30 20:55:54 UTC
HMDB IDHMDB0000011
Secondary Accession Numbers
  • HMDB0000357
  • HMDB00011
  • HMDB00357
Metabolite Identification
Common Name3-Hydroxybutyric acid
Description
Structure
Thumb
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0000011 (3-Hydroxybutyric acid)

  Mrv1652303252017492D          

  7  6  0  0  0  0            999 V2000
   12.3700  -12.1081    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    9.5121  -12.1081    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   10.2266  -10.8705    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   11.6556  -11.6956    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
   10.9411  -12.1081    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   11.6556  -10.8705    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   10.2266  -11.6956    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  2  7  1  0  0  0  0
  3  7  2  0  0  0  0
  4  5  1  0  0  0  0
  5  7  1  0  0  0  0
  1  4  1  0  0  0  0
  4  6  1  1  0  0  0
M  END
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3D MOL for HMDB0000011 (3-Hydroxybutyric acid)

HMDB0000011
     RDKit          3D
3-Hydroxybutyric acid
 15 14  0  0  0  0  0  0  0  0999 V2000
   -1.7425   -0.4236    0.2662 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7444    0.6456    0.0230 C   0  0  1  0  0  0  0  0  0  0  0  0
   -0.4149    1.3086    1.2191 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.5104    0.2340   -0.6764 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.3087   -0.7914   -0.0024 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7814   -1.8855    0.3046 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.6465   -0.5912    0.3127 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6225    0.0533    0.7702 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0681   -0.8741   -0.6781 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3154   -1.2104    0.9171 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2441    1.4129   -0.6506 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.3665    0.8920    1.6687 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.1227    1.1704   -0.8163 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.2318   -0.1403   -1.7054 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.1839    0.1997   -0.0514 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  2  0
  5  7  1  0
  1  8  1  0
  1  9  1  0
  1 10  1  0
  2 11  1  6
  3 12  1  0
  4 13  1  0
  4 14  1  0
  7 15  1  0
M  END
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3D SDF for HMDB0000011 (3-Hydroxybutyric acid)

  Mrv1652303252017492D          

  7  6  0  0  0  0            999 V2000
   12.3700  -12.1081    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    9.5121  -12.1081    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   10.2266  -10.8705    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   11.6556  -11.6956    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
   10.9411  -12.1081    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   11.6556  -10.8705    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   10.2266  -11.6956    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  2  7  1  0  0  0  0
  3  7  2  0  0  0  0
  4  5  1  0  0  0  0
  5  7  1  0  0  0  0
  1  4  1  0  0  0  0
  4  6  1  1  0  0  0
M  END
> <DATABASE_ID>
HMDB0000011

> <DATABASE_NAME>
hmdb

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

> <INCHI_IDENTIFIER>
InChI=1S/C4H8O3/c1-3(5)2-4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m1/s1

> <INCHI_KEY>
WHBMMWSBFZVSSR-GSVOUGTGSA-N

> <FORMULA>
C4H8O3

> <MOLECULAR_WEIGHT>
104.1045

> <EXACT_MASS>
104.047344122

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
15

> <JCHEM_AVERAGE_POLARIZABILITY>
9.959788970081753

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
2

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
(3R)-3-hydroxybutanoic acid

> <ALOGPS_LOGP>
-0.50

> <JCHEM_LOGP>
-0.38700599933333335

> <ALOGPS_LOGS>
0.71

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA>
15.695695082377142

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.406291419798768

> <JCHEM_PKA_STRONGEST_BASIC>
-2.6205133689113183

> <JCHEM_POLAR_SURFACE_AREA>
57.53

> <JCHEM_REFRACTIVITY>
23.464100000000002

> <JCHEM_ROTATABLE_BOND_COUNT>
2

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
5.39e+02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
(-)-3-hydroxybutyric acid

> <JCHEM_VEBER_RULE>
0

$$$$
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3D-SDF for HMDB0000011 (3-Hydroxybutyric acid)

HMDB0000011
     RDKit          3D
3-Hydroxybutyric acid
 15 14  0  0  0  0  0  0  0  0999 V2000
   -1.7425   -0.4236    0.2662 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7444    0.6456    0.0230 C   0  0  1  0  0  0  0  0  0  0  0  0
   -0.4149    1.3086    1.2191 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.5104    0.2340   -0.6764 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.3087   -0.7914   -0.0024 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7814   -1.8855    0.3046 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.6465   -0.5912    0.3127 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6225    0.0533    0.7702 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0681   -0.8741   -0.6781 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3154   -1.2104    0.9171 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2441    1.4129   -0.6506 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.3665    0.8920    1.6687 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.1227    1.1704   -0.8163 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.2318   -0.1403   -1.7054 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.1839    0.1997   -0.0514 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  5  6  2  0
  5  7  1  0
  1  8  1  0
  1  9  1  0
  1 10  1  0
  2 11  1  6
  3 12  1  0
  4 13  1  0
  4 14  1  0
  7 15  1  0
M  END
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PDB for HMDB0000011 (3-Hydroxybutyric acid)

HEADER    PROTEIN                                 25-MAR-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   25-MAR-20         0                                  
HETATM    1  O   UNK     0      23.091 -22.602   0.000  0.00  0.00           O+0
HETATM    2  O   UNK     0      17.756 -22.602   0.000  0.00  0.00           O+0
HETATM    3  O   UNK     0      19.090 -20.292   0.000  0.00  0.00           O+0
HETATM    4  C   UNK     0      21.757 -21.832   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0      20.423 -22.602   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0      21.757 -20.292   0.000  0.00  0.00           C+0
HETATM    7  C   UNK     0      19.090 -21.832   0.000  0.00  0.00           C+0
CONECT    1    4                                                            
CONECT    2    7                                                            
CONECT    3    7                                                            
CONECT    4    5    1    6                                                  
CONECT    5    4    7                                                       
CONECT    6    4                                                            
CONECT    7    2    3    5                                                  
MASTER        0    0    0    0    0    0    0    0    7    0   12    0
END
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3D PDB for HMDB0000011 (3-Hydroxybutyric acid)

COMPND    HMDB0000011
HETATM    1  C1  UNL     1      -1.743  -0.424   0.266  1.00  0.00           C  
HETATM    2  C2  UNL     1      -0.744   0.646   0.023  1.00  0.00           C  
HETATM    3  O1  UNL     1      -0.415   1.309   1.219  1.00  0.00           O  
HETATM    4  C3  UNL     1       0.510   0.234  -0.676  1.00  0.00           C  
HETATM    5  C4  UNL     1       1.309  -0.791  -0.002  1.00  0.00           C  
HETATM    6  O2  UNL     1       0.781  -1.886   0.305  1.00  0.00           O  
HETATM    7  O3  UNL     1       2.647  -0.591   0.313  1.00  0.00           O  
HETATM    8  H1  UNL     1      -2.623   0.053   0.770  1.00  0.00           H  
HETATM    9  H2  UNL     1      -2.068  -0.874  -0.678  1.00  0.00           H  
HETATM   10  H3  UNL     1      -1.315  -1.210   0.917  1.00  0.00           H  
HETATM   11  H4  UNL     1      -1.244   1.413  -0.651  1.00  0.00           H  
HETATM   12  H5  UNL     1       0.366   0.892   1.669  1.00  0.00           H  
HETATM   13  H6  UNL     1       1.123   1.170  -0.816  1.00  0.00           H  
HETATM   14  H7  UNL     1       0.232  -0.140  -1.705  1.00  0.00           H  
HETATM   15  H8  UNL     1       3.184   0.200  -0.051  1.00  0.00           H  
CONECT    1    2    8    9   10
CONECT    2    3    4   11
CONECT    3   12
CONECT    4    5   13   14
CONECT    5    6    6    7
CONECT    7   15
END
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SMILES for HMDB0000011 (3-Hydroxybutyric acid)

C[C@@H](O)CC(O)=O
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INCHI for HMDB0000011 (3-Hydroxybutyric acid)

InChI=1S/C4H8O3/c1-3(5)2-4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m1/s1
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View in JSmolView NMR AssignmentsView Stereo Labels
Synonyms
Chemical FormulaC4H8O3
Average Molecular Weight104.1045
Monoisotopic Molecular Weight104.047344122
IUPAC Name(3R)-3-hydroxybutanoic acid
Traditional Name(-)-3-hydroxybutyric acid
CAS Registry Number625-72-9
SMILES
C[C@@H](O)CC(O)=O
InChI Identifier
InChI=1S/C4H8O3/c1-3(5)2-4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m1/s1
InChI KeyWHBMMWSBFZVSSR-GSVOUGTGSA-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.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassHydroxy acids and derivatives
Sub ClassBeta hydroxy acids and derivatives
Direct ParentBeta hydroxy acids and derivatives
Alternative Parents
Substituents
  • Short-chain hydroxy acid
  • Beta-hydroxy acid
  • Fatty 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 FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Health effect
Disposition
Biological locationSource
Process
Naturally occurring process
Role
Biological roleIndustrial application
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point49 - 50 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
Abnormal Concentrations
Associated Disorders and Diseases
Disease References
Diabetes mellitus type 2
  1. Sheikh-Ali M, Karon BS, Basu A, Kudva YC, Muller LA, Xu J, Schwenk WF, Miles JM: Can serum beta-hydroxybutyrate be used to diagnose diabetic ketoacidosis? Diabetes Care. 2008 Apr;31(4):643-7. doi: 10.2337/dc07-1683. Epub 2008 Jan 9. [PubMed:18184896 ]
  2. Tasker RC, Lutman D, Peters MJ: Hyperventilation in severe diabetic ketoacidosis. Pediatr Crit Care Med. 2005 Jul;6(4):405-11. [PubMed:15982426 ]
  3. Bales JR, Higham DP, Howe I, Nicholson JK, Sadler PJ: Use of high-resolution proton nuclear magnetic resonance spectroscopy for rapid multi-component analysis of urine. Clin Chem. 1984 Mar;30(3):426-32. [PubMed:6321058 ]
  4. del Carmen Crespillo M, Olveira G, de Adana MS, Rojo-Martinez G, Garcia-Aleman J, Olvera P, Soriguer F, Munoz A: Metabolic effects of an enteral nutrition formula for diabetes: comparison with standard formulas in patients with type 1 diabetes. Clin Nutr. 2003 Oct;22(5):483-7. [PubMed:14512036 ]
Obesity
  1. Vice E, Privette JD, Hickner RC, Barakat HA: Ketone body metabolism in lean and obese women. Metabolism. 2005 Nov;54(11):1542-5. [PubMed:16253646 ]
Pancreatic cancer
  1. OuYang D, Xu J, Huang H, Chen Z: Metabolomic profiling of serum from human pancreatic cancer patients using 1H NMR spectroscopy and principal component analysis. Appl Biochem Biotechnol. 2011 Sep;165(1):148-54. doi: 10.1007/s12010-011-9240-0. Epub 2011 Apr 20. [PubMed:21505807 ]
  2. Zhang L, Jin H, Guo X, Yang Z, Zhao L, Tang S, Mo P, Wu K, Nie Y, Pan Y, Fan D: Distinguishing pancreatic cancer from chronic pancreatitis and healthy individuals by (1)H nuclear magnetic resonance-based metabonomic profiles. Clin Biochem. 2012 Sep;45(13-14):1064-9. doi: 10.1016/j.clinbiochem.2012.05.012. Epub 2012 May 19. [PubMed:22613268 ]
Colorectal cancer
  1. 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 ]
  2. 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 ]
Schizophrenia
  1. Fukushima T, Iizuka H, Yokota A, Suzuki T, Ohno C, Kono Y, Nishikiori M, Seki A, Ichiba H, Watanabe Y, Hongo S, Utsunomiya M, Nakatani M, Sadamoto K, Yoshio T: Quantitative analyses of schizophrenia-associated metabolites in serum: serum D-lactate levels are negatively correlated with gamma-glutamylcysteine in medicated schizophrenia patients. PLoS One. 2014 Jul 8;9(7):e101652. doi: 10.1371/journal.pone.0101652. eCollection 2014. [PubMed:25004141 ]
  2. 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 ]
  3. Cai HL, Li HD, Yan XZ, Sun B, Zhang Q, Yan M, Zhang WY, Jiang P, Zhu RH, Liu YP, Fang PF, Xu P, Yuan HY, Zhang XH, Hu L, Yang W, Ye HS: Metabolomic analysis of biochemical changes in the plasma and urine of first-episode neuroleptic-naive schizophrenia patients after treatment with risperidone. J Proteome Res. 2012 Aug 3;11(8):4338-50. doi: 10.1021/pr300459d. Epub 2012 Jul 26. [PubMed:22800120 ]
Early preeclampsia
  1. 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
  1. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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
  1. 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 ]
2-Ketoglutarate dehydrogenase complex deficiency
  1. Guffon N, Lopez-Mediavilla C, Dumoulin R, Mousson B, Godinot C, Carrier H, Collombet JM, Divry P, Mathieu M, Guibaud P: 2-Ketoglutarate dehydrogenase deficiency, a rare cause of primary hyperlactataemia: report of a new case. J Inherit Metab Dis. 1993;16(5):821-30. [PubMed:8295396 ]
3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency
  1. Morris AA, Lascelles CV, Olpin SE, Lake BD, Leonard JV, Quant PA: Hepatic mitochondrial 3-hydroxy-3-methylglutaryl-coenzyme a synthase deficiency. Pediatr Res. 1998 Sep;44(3):392-6. doi: 10.1203/00006450-199809000-00021. [PubMed:9727719 ]
3-Hydroxyacyl-CoA dehydrogenase deficiency
  1. Hsu BY, Kelly A, Thornton PS, Greenberg CR, Dilling LA, Stanley CA: Protein-sensitive and fasting hypoglycemia in children with the hyperinsulinism/hyperammonemia syndrome. J Pediatr. 2001 Mar;138(3):383-9. doi: 10.1067/mpd.2001.111818. [PubMed:11241047 ]
  2. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
  3. Popa FI, Perlini S, Teofoli F, Degani D, Funghini S, La Marca G, Rinaldo P, Vincenzi M, Antoniazzi F, Boner A, Camilot M: 3-hydroxyacyl-coenzyme a dehydrogenase deficiency: identification of a new mutation causing hyperinsulinemic hypoketotic hypoglycemia, altered organic acids and acylcarnitines concentrations. JIMD Rep. 2012;2:71-7. doi: 10.1007/8904_2011_50. Epub 2011 Sep 6. [PubMed:23430856 ]
Long-chain-3-hydroxyacyl CoA dehydrogenase deficiency
  1. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
Short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency
  1. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
Ketotic hypoglycemia
  1. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
  1. Zschocke J, Penzien JM, Bielen R, Casals N, Aledo R, Pie J, Hoffmann GF, Hegardt FG, Mayatepek E: The diagnosis of mitochondrial HMG-CoA synthase deficiency. J Pediatr. 2002 Jun;140(6):778-80. doi: 10.1067/mpd.2002.123854. [PubMed:12072887 ]
Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency
  1. Benkert AR, Young M, Robinson D, Hendrickson C, Lee PA, Strauss KA: Severe Salt-Losing 3beta-Hydroxysteroid Dehydrogenase Deficiency: Treatment and Outcomes of HSD3B2 c.35G>A Homozygotes. J Clin Endocrinol Metab. 2015 Aug;100(8):E1105-15. doi: 10.1210/jc.2015-2098. Epub 2015 Jun 16. [PubMed:26079780 ]
Pyruvate dehydrogenase phosphatase deficiency
  1. Robinson BH, Sherwood WG: Pyruvate dehydrogenase phosphatase deficiency: a cause of congenital chronic lactic acidosis in infancy. Pediatr Res. 1975 Dec;9(12):935-9. doi: 10.1203/00006450-197512000-00015. [PubMed:172850 ]
Carnitine palmitoyltransferase I deficiency
  1. Olpin SE, Allen J, Bonham JR, Clark S, Clayton PT, Calvin J, Downing M, Ives K, Jones S, Manning NJ, Pollitt RJ, Standing SJ, Tanner MS: Features of carnitine palmitoyltransferase type I deficiency. J Inherit Metab Dis. 2001 Feb;24(1):35-42. [PubMed:11286380 ]
Anoxia
  1. Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed:8579834 ]
Meningitis
  1. Subramanian A, Gupta A, Saxena S, Gupta A, Kumar R, Nigam A, Kumar R, Mandal SK, Roy R: Proton MR CSF analysis and a new software as predictors for the differentiation of meningitis in children. NMR Biomed. 2005 Jun;18(4):213-25. [PubMed:15627241 ]
Ulcerative colitis
  1. Azario I, Pievani A, Del Priore F, Antolini L, Santi L, Corsi A, Cardinale L, Sawamoto K, Kubaski F, Gentner B, Bernardo ME, Valsecchi MG, Riminucci M, Tomatsu S, Aiuti A, Biondi A, Serafini M: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I. Sci Rep. 2017 Aug 25;7(1):9473. doi: 10.1038/s41598-017-09958-9. [PubMed:28842642 ]
Crohn's disease
  1. Azario I, Pievani A, Del Priore F, Antolini L, Santi L, Corsi A, Cardinale L, Sawamoto K, Kubaski F, Gentner B, Bernardo ME, Valsecchi MG, Riminucci M, Tomatsu S, Aiuti A, Biondi A, Serafini M: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I. Sci Rep. 2017 Aug 25;7(1):9473. doi: 10.1038/s41598-017-09958-9. [PubMed:28842642 ]
Alzheimer's disease
  1. 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
  1. 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
  1. 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 ]
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Medium Chain Acyl-CoA Dehydrogenase Deficiency
  1. Tserng KY, Jin SJ, Kerr DS, Hoppel CL: Abnormal urinary excretion of unsaturated dicarboxylic acids in patients with medium-chain acyl-CoA dehydrogenase deficiency. J Lipid Res. 1990 May;31(5):763-71. [PubMed:2380628 ]
  2. Gregersen N, Kolvraa S, Rasmussen K, Mortensen PB, Divry P, David M, Hobolth N: General (medium-chain) acyl-CoA dehydrogenase deficiency (non-ketotic dicarboxylic aciduria): quantitative urinary excretion pattern of 23 biologically significant organic acids in three cases. Clin Chim Acta. 1983 Aug 15;132(2):181-91. [PubMed:6616873 ]
  3. Duran M, De Klerk JB, Wadman SK, Bruinvis L, Ketting D: The differential diagnosis of dicarboxylic aciduria. J Inherit Metab Dis. 1984;7 Suppl 1:48-51. [PubMed:6434845 ]
  4. Clayton PT, Eaton S, Aynsley-Green A, Edginton M, Hussain K, Krywawych S, Datta V, Malingre HE, Berger R, van den Berg IE: Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion. J Clin Invest. 2001 Aug;108(3):457-65. [PubMed:11489939 ]
Fumarase deficiency
  1. Bastug O, Kardas F, Ozturk MA, Halis H, Memur S, Korkmaz L, Tag Z, Gunes T: A rare cause of opistotonus; fumaric aciduria: The first case presentation in Turkey. Turk Pediatri Ars. 2014 Mar 1;49(1):74-6. doi: 10.5152/tpa.2014.442. eCollection 2014 Mar. [PubMed:26078636 ]
3-Hydroxyisobutyric acid dehydrogenase deficiency
  1. Ko FJ, Nyhan WL, Wolff J, Barshop B, Sweetman L: 3-Hydroxyisobutyric aciduria: an inborn error of valine metabolism. Pediatr Res. 1991 Oct;30(4):322-6. doi: 10.1203/00006450-199110000-00006. [PubMed:1956714 ]
3-Hydroxydicarboxylic aciduria
  1. Tserng KY, Jin SJ, Kerr DS, Hoppel CL: Urinary 3-hydroxydicarboxylic acids in pathophysiology of metabolic disorders with dicarboxylic aciduria. Metabolism. 1991 Jul;40(7):676-82. [PubMed:1870421 ]
Pyruvate carboxylase deficiency
  1. Habarou F, Brassier A, Rio M, Chretien D, Monnot S, Barbier V, Barouki R, Bonnefont JP, Boddaert N, Chadefaux-Vekemans B, Le Moyec L, Bastin J, Ottolenghi C, de Lonlay P: Pyruvate carboxylase deficiency: An underestimated cause of lactic acidosis. Mol Genet Metab Rep. 2014 Nov 28;2:25-31. doi: 10.1016/j.ymgmr.2014.11.001. eCollection 2015 Mar. [PubMed:28649521 ]
Associated OMIM IDs
  • 125853 (Diabetes mellitus type 2)
  • 601665 (Obesity)
  • 260350 (Pancreatic cancer)
  • 114500 (Colorectal cancer)
  • 181500 (Schizophrenia)
  • 203740 (2-Ketoglutarate dehydrogenase complex deficiency)
  • 605911 (3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency)
  • 231530 (3-Hydroxyacyl-CoA dehydrogenase deficiency)
  • 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
  • 201810 (Adrenal hyperplasia, congenital, due to 3-beta-hydroxysteroid dehydrogenase 2 deficiency)
  • 608782 (Pyruvate dehydrogenase phosphatase deficiency)
  • 255120 (Carnitine palmitoyltransferase I deficiency)
  • 266600 (Crohn's disease)
  • 104300 (Alzheimer's disease)
  • 600274 (Frontotemporal dementia)
  • 610247 (Eosinophilic esophagitis)
  • 201450 (Medium Chain Acyl-CoA Dehydrogenase Deficiency)
  • 606812 (Fumarase deficiency)
  • 266150 (Pyruvate carboxylase deficiency)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB021869
KNApSAcK IDNot Available
Chemspider ID83181
KEGG Compound IDC01089
BioCyc IDCPD-335
BiGG ID36784
Wikipedia LinkBeta-Hydroxybutyric_acid
METLIN IDNot Available
PubChem Compound92135
PDB IDNot Available
ChEBI ID17066
Food Biomarker OntologyNot Available
VMH IDBHB
MarkerDB IDMDB00000005
Good Scents IDNot Available
References
Synthesis ReferenceLe Sann, Christine; Munoz, Dulce M.; Saunders, Natalie; Simpson, Thomas J.; Smith, David I.; Soulas, Florilene; Watts, Paul; Willis, Christine L. Assembly intermediates in polyketide biosynthesis: enantioselective syntheses of b-hydroxycarbonyl compounds. Org Biomol Chem. 2005 May 7;3(9):1719-28. Epub 2005 Mar 31. Pubmed: 15858656
Material Safety Data Sheet (MSDS)Not Available
General References

Enzymes

Enzyme Details
1. 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
3-Hydroxybutyric acid → 3-(sulfooxy)butanoic aciddetails
Enzyme Details
2. 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
3-Hydroxybutyric acid → 6-[(1-carboxypropan-2-yl)oxy]-3,4,5-trihydroxyoxane-2-carboxylic aciddetails
3-Hydroxybutyric acid → 3,4,5-trihydroxy-6-[(3-hydroxybutanoyl)oxy]oxane-2-carboxylic aciddetails
Enzyme Details
3. D-beta-hydroxybutyrate dehydrogenase, mitochondrial
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
BDH1
Uniprot ID:
Q02338
Molecular weight:
38156.77
Reactions
3-Hydroxybutyric acid + NAD → Acetoacetic acid + NADHdetails
3-Hydroxybutyric acid + NAD → Acetoacetic acid + NADH + Hydrogen Iondetails
Enzyme Details
4. 3-hydroxybutyrate dehydrogenase type 2
General function:
Involved in oxidoreductase activity
Specific function:
Dehydrogenase that mediates the formation of 2,5-dihydroxybenzoic acid (2,5-DHBA), a siderophore that shares structural similarities with bacterial enterobactin and associates with LCN2, thereby playing a key role in iron homeostasis and transport. Also acts as a 3-hydroxybutyrate dehydrogenase (By similarity).
Gene Name:
BDH2
Uniprot ID:
Q9BUT1
Molecular weight:
26723.57
Reactions
3-Hydroxybutyric acid + NAD → Acetoacetic acid + NADHdetails
3-Hydroxybutyric acid + NAD → Acetoacetic acid + NADH + Hydrogen Iondetails
Enzyme Details
5. Novel protein similar to 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (Hydroxymethylglutaricaciduria)
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
DKFZp434G1411
Uniprot ID:
Q9NT06
Molecular weight:
16899.1
References
  1. Gibson KM, Lee CF, Kamali V, Johnston K, Beaudet AL, Craigen WJ, Powell BR, Schwartz R, Tsai MY, Tuchman M: 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency as detected by radiochemical assay in cell extracts by thin-layer chromatography, and identification of three new cases. Clin Chem. 1990 Feb;36(2):297-303. [PubMed:2302772 ]