Human Metabolome Database Version 3.5

Showing metabocard for (R)-3-Hydroxybutyric acid (HMDB00011)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-04-19 16:20:42 -0600
HMDB ID HMDB00011
Secondary Accession Numbers None
Metabolite Identification
Common Name (R)-3-Hydroxybutyric acid
Description (R)-3-Hydroxybutyric acid is a butyric acid substituted with a hydroxyl group in the beta or 3 position. 3-hydroxybutyric acid, or beta-hydroxybutyrate, is involved in the synthesis and degradation of ketone bodies. Like the other ketone bodies (acetoacetate and acetone), levels of beta-hydroxybutyrate are raised in the blood and urine in ketosis. Beta-hydroxybutyrate is a typical partial-degradation product of branched-chain amino acids (primarily valine) released from muscle for hepatic and renal gluconeogenesis This acid is metabolized by 3-hydroxybutyrate dehydrogenase (catalyzes the oxidation of D-3-hydroxybutyrate to form acetoacetate, using NAD+ as an electron acceptor). The enzyme functions in nervous tissues and muscles, enabling the use of circulating hydroxybutyrate as a fuel. In the liver mitochondrial matrix, the enzyme can also catalyze the reverse reaction, a step in ketogenesis. 3-Hydroxybutyric acid is a chiral compound having two enantiomers, D-3-hydroxybutyric acid and L-3-hydroxybutyric acid. In humans, beta-hydroxybutyrate is synthesized in the liver from acetyl-CoA, and can be used as an energy source by the brain when blood glucose is low. It can also be used for the synthesis of biodegradable plastics (Wikipedia).
Structure Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. (R)-(-)-b-Hydroxybutyrate
  2. (R)-(-)-b-Hydroxybutyric acid
  3. (R)-(-)-beta-Hydroxybutyrate
  4. (R)-(-)-beta-Hydroxybutyric acid
  5. (R)-3-Hydroxybutanoate
  6. (R)-3-Hydroxybutanoic acid
  7. (R)-3-Hydroxybutyrate
  8. 3-D-Hydroxybutyrate
  9. 3-D-Hydroxybutyric acid
  10. 3-delta-Hydroxybutyrate
  11. 3-delta-Hydroxybutyric acid
  12. BHIB
  13. D-(-)-3-Hydroxybutyrate
  14. D-3-Hydroxybutyrate
  15. D-3-Hydroxybutyric acid
  16. D-beta-Hydroxybutyrate
  17. delta-(-)-3-Hydroxybutyrate
  18. delta-3-Hydroxybutyrate
  19. delta-3-Hydroxybutyric acid
  20. delta-beta-Hydroxybutyrate
Chemical Formula C4H8O3
Average Molecular Weight 104.1045
Monoisotopic Molecular Weight 104.047344122
IUPAC Name (3R)-3-hydroxybutanoic acid
Traditional IUPAC Name (-)-3-hydroxybutyric acid
CAS Registry Number 625-72-9
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 Key WHBMMWSBFZVSSR-GSVOUGTGSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Organic Acids and Derivatives
Class Hydroxy Acids and Derivatives
Sub Class Beta Hydroxy Acids and Derivatives
Other Descriptors
  • 3-hydroxy monocarboxylic acid(ChEBI)
  • Aliphatic Acyclic Compounds
  • Hydroxy fatty acids(Lipidmaps)
  • Organic Compounds
  • Straight Chain Fatty Acids
  • hydroxy fatty acid(ChEBI)
  • hydroxybutyric acid(ChEBI)
  • short-chain fatty acid(ChEBI)
  • straight-chain saturated fatty acid(ChEBI)
Substituents
  • Carboxylic Acid
  • Secondary Alcohol
  • Short Chain Hydroxy Acid
Direct Parent Beta Hydroxy Acids and Derivatives
Ontology
Status Detected and Quantified
Origin
  • Endogenous
Biofunction
  • Component of Butanoate metabolism
Application Not Available
Cellular locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point 49 - 50 °C Not Available
Boiling Point Not Available Not Available
Water Solubility Not Available Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 539 g/L ALOGPS
LogP -0.50 ALOGPS
LogP -0.39 ChemAxon
LogS 0.71 ALOGPS
pKa (strongest acidic) 4.41 ChemAxon
pKa (strongest basic) -2.6 ChemAxon
Hydrogen Acceptor Count 3 ChemAxon
Hydrogen Donor Count 2 ChemAxon
Polar Surface Area 57.53 A2 ChemAxon
Rotatable Bond Count 2 ChemAxon
Refractivity 23.46 ChemAxon
Polarizability 9.96 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge -1 ChemAxon
Spectra
1H NMR Spectrum
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum Quattro_QQQ 40
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 10
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 20
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 30
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 40
MS/MS Spectrum LC-ESI-QQ (API3000, Applied Biosystems) 50
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location Not Available
Pathways
Name SMPDB Link KEGG Link
Ketone Body Metabolism SMP00071 map00072 Link_out
Normal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified
36 +/- 20 uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
86 +/- 53 uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
38 +/- 31 uM Adult (>18 years old) Both Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
286 (207-365) uM Adult (>18 years old) Both Normal
Urine Detected and Quantified
3.4 umol/mmol creatinine Adult (>18 years old) Not Specified Normal
Urine Detected and Quantified
48.0 (0.00-200.0) umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
3.6 (1.3-6.4) umol/mmol creatinine Adult (>18 years old) Both Normal
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified 1500.0 +/- 100.0 uM Children (1-13 year old) Both Comment Diabetes
Blood Detected and Quantified 7700.0 +/- 300.0 uM Children (1-13 year old) Both Comment Diabetes
Blood Detected and Quantified 1400.0 +/- 100.0 uM Adult (>18 years old) Both Comment Diabetes
Blood Detected and Quantified 7700.0 +/- 200.0 uM Adult (>18 years old) Both Comment Diabetes
Cerebrospinal Fluid (CSF) Detected and Quantified 430 (359-501) uM Adult (>18 years old) Both Comment Meningitis
Cerebrospinal Fluid (CSF) Detected and Quantified 6360 (5350-7370) uM Adult (>18 years old) Both Comment Diabetes
Urine Detected and Quantified 224 umol/mmol creatinine Adult (>18 years old) Both Diabetes
Associated Disorders and Diseases
Disease References
Diabetes mellitus type 2
  • 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. Epub 2008 Jan 9. Pubmed: 18184896 Link_out
  • Tasker RC, Lutman D, Peters MJ: Hyperventilation in severe diabetic ketoacidosis. Pediatr Crit Care Med. 2005 Jul;6(4):405-11. Pubmed: 15982426 Link_out
  • 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 Link_out
      Meningitis
      • 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 Link_out
          Associated OMIM IDs
          • 125853 Link_out (Diabetes mellitus type 2)
          DrugBank ID Not Available
          DrugBank Metabolite ID Not Available
          Phenol Explorer Compound ID Not Available
          Phenol Explorer Metabolite ID Not Available
          FoodDB ID FDB021869
          KNApSAcK ID Not Available
          Chemspider ID 83181 Link_out
          KEGG Compound ID C01089 Link_out
          BioCyc ID CPD-335 Link_out
          BiGG ID 36784 Link_out
          Wikipedia Link Not Available
          NuGOwiki Link HMDB00011 Link_out
          Metagene Link HMDB00011 Link_out
          METLIN ID Not Available
          PubChem Compound 92135 Link_out
          PDB ID 3HR Link_out
          ChEBI ID 17066 Link_out
          References
          Synthesis Reference Le 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
          Material Safety Data Sheet (MSDS) Download (PDF)
          General References
          1. Pan JW, Rothman TL, Behar KL, Stein DT, Hetherington HP: Human brain beta-hydroxybutyrate and lactate increase in fasting-induced ketosis. J Cereb Blood Flow Metab. 2000 Oct;20(10):1502-7. Pubmed: 11043913 Link_out
          2. Pan JW, Telang FW, Lee JH, de Graaf RA, Rothman DL, Stein DT, Hetherington HP: Measurement of beta-hydroxybutyrate in acute hyperketonemia in human brain. J Neurochem. 2001 Nov;79(3):539-44. Pubmed: 11701757 Link_out
          3. Plecko B, Stoeckler-Ipsiroglu S, Schober E, Harrer G, Mlynarik V, Gruber S, Moser E, Moeslinger D, Silgoner H, Ipsiroglu O: Oral beta-hydroxybutyrate supplementation in two patients with hyperinsulinemic hypoglycemia: monitoring of beta-hydroxybutyrate levels in blood and cerebrospinal fluid, and in the brain by in vivo magnetic resonance spectroscopy. Pediatr Res. 2002 Aug;52(2):301-6. Pubmed: 12149510 Link_out
          4. Raunio RP, Leivo PV, Kuusinen AM: Bioluminescent assay of D-3-hydroxybutyrate in serum. J Biolumin Chemilumin. 1986 Jun;1(1):11-4. Pubmed: 3503522 Link_out
          5. Bernardi, R. et al., Chem. Commun., 1984, 460, (resoln)
          6. Encyclopedia of Food and Color Additives, (ed. Burdock, G.A.), CRC Press, 1997, 994-995, (Et ester)
          7. Fenaroli's Handbook of Flavor Ingredients, 3rd edn., (ed. Burdock, G.A.), CRC Press, 1995, 1, 238, (Et ester)
          8. Forsyth, W.G.C. et al., Nature (London), 1958, 182, 800, (isol)
          9. Gottschalk, G. et al., Nature (London), 1965, 205, 308
          10. Krajewski, D. et al., Phytochemistry, 1997, 45, 1627, (ester glucosides)
          11. Levene, P.A. et al., J. Biol. Chem., 1926, 68, 418, (abs config)
          12. Lewis, R.J., Sax's Dangerous Properties of Industrial Materials, 8th edn., Van Nostrand Reinhold, 1992, MEF500 MKP250
          13. Marchessault, R.H. et al., Makromol. Chem., Macromol. Symp., 1988, 19, 235, (rev, polymers)
          14. Muller, H.-M. et al., Angew. Chem., Int. Ed., 1993, 32, 477, (rev, polymer)
          15. Org. Synth., 1993, 71, 39, (synth, acid, Meester, pmr, ir)
          16. Quang DN, Hashimoto T, Toyota M, Asakawa Y. Occurrence of a high concentration of spider pheromones in the ascomycete fungus Hypoxylon truncatum. J Nat Prod. 2003 Dec;66(12):1613-4.
          17. Schulz S, Toft S. Identification of a sex pheromone from a spider. Science. 1993 Jun 11;260(5114):1635-7.
          18. Seebach, D. et al., Helv. Chim. Acta, 1982, 65, 495-503, (esters, synth)
          19. Seebach, D. et al., Helv. Chim. Acta, 1988, 71, 155, (synth, dimer)
          20. Wipf, B. et al., Helv. Chim. Acta, 1983, 66, 485

          Enzymes
          Name: D-beta-hydroxybutyrate dehydrogenase, mitochondrial
          Reactions:
          (R)-3-Hydroxybutyric acid + NAD unknown Acetoacetic acid + NADH details
          (R)-3-Hydroxybutyric acid + NAD unknown Acetoacetic acid + NADH + Hydrogen Ion details
          Gene Name: BDH1
          Uniprot ID: Q02338 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: 3-hydroxybutyrate dehydrogenase type 2
          Reactions:
          (R)-3-Hydroxybutyric acid + NAD unknown Acetoacetic acid + NADH details
          (R)-3-Hydroxybutyric acid + NAD unknown Acetoacetic acid + NADH + Hydrogen Ion details
          Gene Name: BDH2
          Uniprot ID: Q9BUT1 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA
          Name: Novel protein similar to 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase (Hydroxymethylglutaricaciduria)
          Reactions: Not Available
          Gene Name: DKFZp434G1411
          Uniprot ID: Q9NT06 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA