We are currently updating the database - data may be missing for the next 10 minutes. We apologize for any inconvenience.

Human Metabolome Database Version 2.5

 

Showing metabocard for 3-Hydroxybutyric acid (HMDB00357)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2005-11-16 15:48:42
Update Date 2010-03-10 19:11:00
Accession Number HMDB00357
Secondary Accession Numbers Not Available
Common Name 3-Hydroxybutyric acid
Description 3-Hydroxybutyric acid (or beta-hydroxybutyrate) is a ketone body. Like the other ketone bodies (acetoacetate and acetone), levels of 3-hydroxybutyrate in blood and urine are raised in ketosis. In humans, 3-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. Blood levels of 3-Hydroxybutyric acid levels may be monitored in diabetic patients to look for diabetic ketoacidosis. Persistent mild hyperketonemia is a common finding in newborns. Ketone bodies serve as an indispensable source of energy for extrahepatic tissues, especially the brain and lung of developing mammals. Another important function of ketone bodies is to provide acetoacetyl-CoA and acetyl-CoA for synthesis of cholesterol, fatty acids, and complex lipids. During the early postnatal period, acetoacetate (AcAc) and beta-hydroxybutyrate are preferred over glucose as substrates for synthesis of phospholipids and sphingolipids in accord with requirements for brain growth and myelination. Thus, during the first 2 weeks of postnatal development, when the accumulation of cholesterol and phospholipids accelerates, the proportion of ketone bodies incorporated into these lipids increases. On the other hand, an increased proportion of ketone bodies is utilized for cerebroside synthesis during the period of active myelination. In the lung, AcAc serves better than glucose as a precursor for the synthesis of lung phospholipids. The synthesized lipids, particularly dipalmityl phosphatidylcholine, are incorporated into surfactant, and thus have a potential role in supplying adequate surfactant lipids to maintain lung function during the early days of life. (PMID 3884391)
Synonyms
  1. 3-OH-butyric acid
  2. 3-Hydroxybutanoate
  3. 3-Hydroxybutanoic acid
  4. 3-hydroxybutyric acid
  5. 3-Hydroxybutyrate
  6. DL-3-Hydroxybutyrate
  7. DL-3-Hydroxybutyric acid
  8. b-Hydroxy-n-butyrate
  9. b-Hydroxy-n-butyric acid
  10. b-Hydroxybutanoate
  11. b-Hydroxybutanoic acid
  12. b-Hydroxybutyrate
  13. b-Hydroxybutyric acid
  14. beta-Hydroxy-n-butyrate
  15. beta-Hydroxy-n-butyric acid
  16. beta-Hydroxybutyrate
  17. beta-Hydroxybutyric acid
  18. beta-Hydroxybutanoate
  19. beta-Hydroxybutanoic acid
  20. Biopol
  21. 3-hydroxy-butanoic acid
  22. 3-hydroxy-butanoate
  23. 3-hydroxy-butyric acid
  24. 3-hydroxy-butyrate
  25. beta-Hydroxybuttersaeure
  26. DL-b-Hydroxybutyric acid
  27. DL-beta-Hydroxybutyric acid
Chemical IUPAC Name 3-hydroxybutanoic acid
Chemical Formula C4H8O3
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Organic acids
Class
  • Hydroxy Acids
Sub Class
  • Short chain hydroxy acids
Family
  • Mammalian Metabolite
Species
  • secondary alcohol
  • carboxylic acid
Biofunction
—
Application
—
Source
  • Endogenous
Average Molecular Weight 104.104
Monoisotopic Molecular Weight 104.047340
Isomeric SMILES CC(O)CC(O)=O
Canonical SMILES CC(O)CC(O)=O
KEGG Compound ID C01089 Link Image
BioCyc ID CPD-335 Link Image
BiGG ID Not Available
Wikipedia Link 3-Hydroxybutyric acid Link Image
NuGOwiki Link HMDB00357 Link Image
Metagene Link HMDB00357 Link Image
METLIN ID 125 Link Image
PubChem Compound 441 Link Image
PubChem Substance 2380 Link Image
ChEBI ID 20067 Link Image
CAS Registry Number 300-85-6
InChI Identifier InChI=1/C4H8O3/c1-3(5)2-4(6)7/h3,5H,2H2,1H3,(H,6,7)
Synthesis Reference Yang, Wenling; Ma, Peisheng; Wang, Jianing; Wang, Chunfang; Li, Haixia. Study on the process of chemical synthesis of 3-hydroxybutyric acid. Huaxue Gongcheng (Xi'an, China) (2002), 30(5), 74-78.
Melting Point (Experimental) 46 oC
Experimental Water Solubility 444 mg/mL [HMP experimental] Source: PhysProp
Predicted Water Solubility 539.0 mg/mL [Predicted by ALOGPS]; 1000 mg/mL at 25 oC [MEYLAN,WM et al. (1996)] Calculated using ALOGPS
Physiological Charge -1
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity -0.50 [Predicted by ALOGPS]; -0.5 [Predicted by PubChem via XLOGP]; -0.47 [MEYLAN,WM & HOWARD,PH (1995)] Calculated using ALOGPS
Material Safety Data Sheet (MSDS)
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID Not Available
Experimental 1H NMR Spectrum Download Spectrum
Download FID (Varian)
Show Experimental Conditions Link Image
Experimental 13C NMR Spectrum Download Spectrum
Download FID (Bruker)
Show Experimental Conditions Link Image
Experimental 13C HSQC Spectrum Download Spectrum
Download FID (Bruker)
Show Experimental Conditions Link Image
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
Show Peaklist
Mass Spectrum
Low Energy
Download File
Show Experimental Conditions Link Image
Medium Energy
Download File
Show Experimental Conditions Link Image
High Energy
Download File
Show Experimental Conditions Link Image
Simplified TOCSY Spectrum Show Image
Show Peaklist
BMRB Spectrum Show Image
Show Peaklist
Cellular Location Not Available
Biofluid Location
  • Blood
  • Cellular Cytoplasm
  • Cerebrospinal Fluid
  • Urine
Tissue Location
Tissue References
Brain —
Liver —
Neuron —
Concentrations (Normal)
Biofluid Blood
Value 180.0 (22.0-700.0) uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. [PubMed Link Image]
Biofluid Blood
Value 565.0 +/- 255.0 uM
Age Newborn:0-30 days old
Sex Both
Patient information Normal (3 days) not fasting
Comments Not Available
References
  • Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.
Biofluid Blood
Value 36.0 (13.0-95.0) uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal (not fasting)
Comments Not Available
References
  • Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.
Biofluid Blood
Value 60.0 +/- 20.0 uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Burns SF, Broom DR, Miyashita M, Ueda C, Stensel DJ: Increased postprandial triacylglycerol concentrations following resistance exercise. Med Sci Sports Exerc. 2006 Mar;38(3):527-33. [PubMed Link Image]
Biofluid Blood
Value 40.0 +/- 10.0 uM
Age Adult:>18 yrs old
Sex Both
Patient information Exercise
Comments Not Available
References
  • Burns SF, Broom DR, Miyashita M, Ueda C, Stensel DJ: Increased postprandial triacylglycerol concentrations following resistance exercise. Med Sci Sports Exerc. 2006 Mar;38(3):527-33. [PubMed Link Image]
Biofluid Blood
Value 323.0 +/- 29.0 uM
Age Adult:>18 yrs old
Sex Female
Patient information Lean women
Comments Not Available
References
  • Vice E, Privette JD, Hickner RC, Barakat HA: Ketone body metabolism in lean and obese women. Metabolism. 2005 Nov;54(11):1542-5. [PubMed Link Image]
Biofluid Blood
Value 50.0 (0.0-100.0) uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Darzy KH, Murray RD, Gleeson HK, Pezzoli SS, Thorner MO, Shalet SM: The impact of short-term fasting on the dynamics of 24-hour growth hormone (GH) secretion in patients with severe radiation-induced GH deficiency. J Clin Endocrinol Metab. 2006 Mar;91(3):987-94. Epub 2005 Dec 29. [PubMed Link Image]
Biofluid Blood
Value 100.0 +/- 90.0 uM
Age Adult:>18 yrs old
Sex Female
Patient information Normal pregnancy
Comments Not Available
References
  • Jovanovic L, Metzger BE, Knopp RH, conley MR, Park E, Lee YJ, Simpson JL, Holmes L, Aarons JH, Mills JL: The Diabetes in Early Pregnancy Study: beta-hydroxybutyrate levels in type 1 diabetic pregnancy compared with normal pregnancy. NICHD-Diabetes in Early Pregnancy Study Group (DIEP). National Institute of Child Health and Development. Diabetes Care. 1998 Nov;21(11):1978-84. [PubMed Link Image]
Biofluid Cellular Cytoplasm
Value 1300 (1200-1400) uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Biofluid CSF
Value 46.4 +/- 23.9 uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Geigy Scientific Tables, 8th Rev edition, pp. 165-177. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp., Basel, Switzerland c1981-1992.
Biofluid CSF
Value 50.0 (4.0 - 96.0) uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. [PubMed Link Image]
Biofluid CSF
Value 48.0 +/- 48.0 uM
Age Adult:>18 yrs old
Sex N/A
Patient information Normal
Comments Not Available
References
  • Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. [PubMed Link Image]
Biofluid CSF
Value 34 +/- 31 uM
Age N/A
Sex Both
Patient information Normal
Comments Not Available
References
  • Wishart DS, Lewis MJ, Morrissey JA, Flegel MD, Jeroncic K, Xiong Y, Cheng D, Eisner R, Gautam B, Tzur D, Sawhney S, Bamforth F, Greiner R, Li L: The human cerebrospinal fluid metabolome. J Chromatogr B Analyt Technol Biomed Life Sci. 2008 Aug 15;871(2):164-173. Epub 2008 May 8. [PubMed Link Image]
Biofluid Urine
Value 2.0 (0.5-9.8) umol/mmol creatinine
Age Newborn:0-30 days old
Sex Both
Patient information Normal
Comments Not Available
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 Link Image]
Biofluid Urine
Value 1.4 (0.1-2.7) umol/mmol creatinine
Age Children:1-13 yrs old
Sex Both
Patient information Normal
Comments Not Available
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 Link Image]
Biofluid Urine
Value 1.3 (0.1-5.8) umol/mmol creatinine
Age Adolescent:13-18 yrs old
Sex Both
Patient information Normal
Comments Not Available
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 Link Image]
Biofluid Urine
Value 38.9 +/- 13.9 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Male
Patient information Normal
Comments Not Available
References
  • Psihogios NG, Gazi IF, Elisaf MS, Seferiadis KI, Bairaktari ET: Gender-related and age-related urinalysis of healthy subjects by NMR-based metabonomics. NMR Biomed. 2008 Mar;21(3):195-207. [PubMed Link Image]
Biofluid Urine
Value 32.3 +/- 8.7 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Female
Patient information Normal
Comments Not Available
References
  • Psihogios NG, Gazi IF, Elisaf MS, Seferiadis KI, Bairaktari ET: Gender-related and age-related urinalysis of healthy subjects by NMR-based metabonomics. NMR Biomed. 2008 Mar;21(3):195-207. [PubMed Link Image]
Biofluid Urine
Value 2.60 +/- 3.50 umol/mmol creatinine
Age Infant:0-1 yr old
Sex Both
Patient information Normal
Comments Not Available
References
  • Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [PubMed Link Image]
Biofluid Urine
Value 2.0 (0.6-5.6) umol/mmol creatinine
Age Infant:0-1 yr old
Sex Both
Patient information Normal
Comments Not Available
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 Link Image]
Biofluid Urine
Value 13.0 (1.0-27.0) umol/mmol creatinine
Age Children:1-13 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992.
Biofluid Urine
Value 180.4 +/- 115.00 umol/mmol creatinine
Age Children:1-13 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Concentrations (Abnormal)
Biofluid Blood
Value 235.0 +/- 17.0 uM
Age Adult:>18 yrs old
Sex Female
Condition Obesity
Comments Not Available
References
  • Vice E, Privette JD, Hickner RC, Barakat HA: Ketone body metabolism in lean and obese women. Metabolism. 2005 Nov;54(11):1542-5. [PubMed Link Image]
Biofluid Blood
Value 35.5 +/- 92.2 uM
Age Adult:>18 yrs old
Sex Both
Condition Diabetes mellitus type 2
Comments Type 1
References
  • 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 Link Image]
Biofluid Blood
Value 5700.00 (4300.00-6000.00) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Comments Not Available
References
  • Charles RA, Bee YM, Eng PH, Goh SY: Point-of-care blood ketone testing: screening for diabetic ketoacidosis at the emergency department. Singapore Med J. 2007 Nov;48(11):986-9. [PubMed Link Image]
Biofluid Cellular Cytoplasm
Value 2600 (2500-2700) uM
Age Adult:>18 yrs old
Sex Both
Condition Anoxia
Comments Not Available
References
  • 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 Link Image]
Associated Disorders
Condition References
Anoxia
  • 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 Link Image]
Diabetes mellitus type 2
  • 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 Link Image]
Obesity
  • Vice E, Privette JD, Hickner RC, Barakat HA: Ketone body metabolism in lean and obese women. Metabolism. 2005 Nov;54(11):1542-5. [PubMed Link Image]
OMIM ID
Pathways
Name SMPDB Link KEGG Link
Fatty Acid Biosynthesis SMP00456 Link Image
General References
  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 Link Image]
  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 Link Image]
  3. Redjems-Bennani N, Jeandel C, Lefebvre E, Blain H, Vidailhet M, Gueant JL: Abnormal substrate levels that depend upon mitochondrial function in cerebrospinal fluid from Alzheimer patients. Gerontology. 1998;44(5):300-4. [PubMed Link Image]
  4. Abrahamsson K, Eriksson BO, Holme E, Jodal U, Jonsson A, Lindstedt S: Pivalic acid-induced carnitine deficiency and physical exercise in humans. Metabolism. 1996 Dec;45(12):1501-7. [PubMed Link Image]
  5. Teresinski G, Buszewicz G, Madro R: The influence of ethanol on the level of ketone bodies in hypothermia. Forensic Sci Int. 2002 Jun 25;127(1-2):88-96. [PubMed Link Image]
  6. Nalecz KA, Miecz D, Berezowski V, Cecchelli R: Carnitine: transport and physiological functions in the brain. Mol Aspects Med. 2004 Oct-Dec;25(5-6):551-67. [PubMed Link Image]
  7. 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 Link Image]
  8. Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [PubMed Link Image]
  9. Blomqvist G, Alvarsson M, Grill V, Von Heijne G, Ingvar M, Thorell JO, Stone-Elander S, Widen L, Ekberg K: Effect of acute hyperketonemia on the cerebral uptake of ketone bodies in nondiabetic subjects and IDDM patients. Am J Physiol Endocrinol Metab. 2002 Jul;283(1):E20-8. [PubMed Link Image]
  10. Krotkiewski M: Value of VLCD supplementation with medium chain triglycerides. Int J Obes Relat Metab Disord. 2001 Sep;25(9):1393-400. [PubMed Link Image]
  11. Kamijo T, Indo Y, Souri M, Aoyama T, Hara T, Yamamoto S, Ushikubo S, Rinaldo P, Matsuda I, Komiyama A, Hashimoto T: Medium chain 3-ketoacyl-coenzyme A thiolase deficiency: a new disorder of mitochondrial fatty acid beta-oxidation. Pediatr Res. 1997 Nov;42(5):569-76. [PubMed Link Image]
  12. Geary N, Grotschel H, Scharrer E: Blood metabolites and feeding during postinsulin hypophagia. Am J Physiol. 1982 Sep;243(3):R304-11. [PubMed Link Image]
  13. Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. [PubMed Link Image]
  14. 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 Link Image]
  15. Frenkel G, Peterson RN, Freund M: Oxidative and glycolytic metabolism of semen components by washed guinea pig spermatozoa. Fertil Steril. 1975 Feb;26(2):144-7. [PubMed Link Image]
  16. Wikipedia Link Image
Metabolic Enzymes
  1. D-beta-hydroxybutyrate dehydrogenase, mitochondrial precursor
  2. 3-hydroxybutyrate dehydrogenase type 2
Enzyme 1 [top]
Enzyme 1 ID 5800
Enzyme 1 Name D-beta-hydroxybutyrate dehydrogenase, mitochondrial precursor
Enzyme 1 Synonyms
  1. BDH
  2. 3-hydroxybutyrate dehydrogenase
Enzyme 1 Gene Name BDH1
Enzyme 1 Protein Sequence >D-beta-hydroxybutyrate dehydrogenase, mitochondrial precursor 
MLATRLSRPLSRLPGKTLSACDRENGARRPLLLGSTSFIPIGRRTYASAAEPVGSKAVLV
TGCDSGFGFSLAKHLHSKGFLVFAGCLMKDKGHDGVKELDSLNSDRLRTVQLNVCSSEEV
EKVVEIVRSSLKDPEKGMWGLVNNAGISTFGEVEFTSLETYKQVAEVNLWGTVRMTKSFL
PLIRRAKGRVVNISSMLGRMANPARSPYCITKFGVEAFSDCLRYEMYPLGVKVSVVEPGN
FIAATSLYSPESIQAIAKKMWEELPEVVRKDYGKKYFDEKIAKMETYCSSGSTDTSPVID
AVTHALTATTPYTRYHPMDYYWWLRMQIMTHLPGAISDMIYIR
Enzyme 1 Number of Residues 343
Enzyme 1 Molecular Weight 38158
Enzyme 1 Theoretical pI 9.24
Enzyme 1 GO Classification
Function
  • catalytic activity
  • oxidoreductase activity
Process
  • metabolism
  • physiological process
Component
—
Enzyme 1 General Function Lipid transport and metabolism
Enzyme 1 Specific Function (R)-3-hydroxybutanoate + NAD(+) = acetoacetate + NADH
Enzyme 1 Pathways
Enzyme 1 Reactions
  • (R)-3-hydroxybutanoate + NAD+ = acetoacetate + NADH + H+
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • None
Enzyme 1 Transmembrane Regions
  • None
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 177198 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID Q02338 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name BDH_HUMAN Link Image
Enzyme 1 PDB ID Not Available
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence >1032 bp 
GGCCTGCGGCCTCCGCCACCCGGACGCTTCTCACGGCTCCCAGGAAAAACCCTAAGTGCC
TGTGATAGAGAAAATGGAGCAAGACGCCCACTATTGCTTGGTTCTACTTCCTTTATCCCG
ATTGGCCGTCGGACTTATGCCAGTGCGGCGGAGCCGGTTGGCAGCAAAGCTGTCCTGGTC
ACAGGCTGTGACTCTGGATTTGGGTTCTCATTGGCCAAGCATCTGCATTCAAAAGGCTTC
CTTGTGTTTGCTGGCTGCTTGATGAAGGACAAAGGCCATGATGGGGTCAAGGAGCTGGAC
AGCCTAAACAGTGACCGATTGAGAACCGTCCAGCTCAATGTCTTCAGAAGCGAAGAGGTG
GAGAAAGTGGTGGGAGATTGTCCGTTCGAGCCTGAAGGACCTGAGAAAGGCATGTGGGGG
CTCGTTAACAATGCCGGCATCTCAACGTTCGGGGAGGTGGAGTTCACCAGCCTGGAGACC
TACAAGCAGGTGGCAGAAGTGAACCTTTGGGGCACAGTGCGGATGACGAAATCCTTTCTC
CCCCTCATCCGAAGGGCCAAAGGCCGCGTCGTCAATATCAGCAGCATGCTGGGCCGCATG
GCCAACCCGGCCCGCTCCCCGTACTGCATCACCAAGTTCGGGGTAGAGGCTTTCTCGGAC
TGCCTGCGCTATGAGATGTACCCCCTGGGCGTGAAGGTCAGCGTGGTGGAGCCCGGCAAC
TTCATCGCTGCCACCAGCCTTTACAACCCTGAGAGCATTCAGGCCATCGCCAAGAAGATG
TGGGAGGAGCTGCCTGAGGTCGTGCGCAAGGACTACGGCAAGAAGTACTTTGATGAAAAG
ATCGCCAAGATGGAGACCTACTGCAGCAGTGGCTCCACAGACACGTCCCCTGTCATCGAT
GCTGTCACACACGCCCTGACCGCCACCACCCCCTACACCCGCTACCACCCCATGGACTAC
TACTGGTGGCTGCGAATGCAGATCATGACCCACTTGCCTGGAGCCATCTCCGACATGATC
TACATCCGCTGA
Enzyme 1 GenBank Gene ID M93107 Link Image
Enzyme 1 GeneCard ID BDH1 Link Image
Enzyme 1 GenAtlas ID BDH1 Link Image
Enzyme 1 HGNC ID HGNC:1027 Link Image
Enzyme 1 Chromosome Location 3
Enzyme 1 Locus 3q29
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References
  1. Marks AR, McIntyre JO, Duncan TM, Erdjument-Bromage H, Tempst P, Fleischer S: Molecular cloning and characterization of (R)-3-hydroxybutyrate dehydrogenase from human heart. J Biol Chem. 1992 Aug 5;267(22):15459-63. [PubMed Link Image]
Enzyme 1 Metabolite References Not Available
Enzyme 2 [top]
Enzyme 2 ID 12878
Enzyme 2 Name 3-hydroxybutyrate dehydrogenase type 2
Enzyme 2 Synonyms
  1. R-beta- hydroxybutyrate dehydrogenase
  2. Dehydrogenase/reductase SDR family member 6
  3. Oxidoreductase UCPA
Enzyme 2 Gene Name BDH2
Enzyme 2 Protein Sequence >3-hydroxybutyrate dehydrogenase type 2 
MGRLDGKVIILTAAAQGIGQAAALAFAREGAKVIATDINESKLQELEKYPGIQTRVLDVT
KKKQIDQFANEVERLDVLFNVAGFVHHGTVLDCEEKDWDFSMNLNVRSMYLMIKAFLPKM
LAQKSGNIINMSSVASSVKGVVNRCVYSTTKAAVIGLTKSVAADFIQQGIRCNCVCPGTV
DTPSLQERIQARGNPEEARNDFLKRQKTGRFATAEEIAMLCVYLASDESAYVTGNPVIID
GGWSL
Enzyme 2 Number of Residues 245
Enzyme 2 Molecular Weight 26724
Enzyme 2 Theoretical pI Not Available
Enzyme 2 GO Classification
Function
  • catalytic activity
  • oxidoreductase activity
Process
  • metabolism
  • physiological process
Component
—
Enzyme 2 General Function Lipid transport and metabolism
Enzyme 2 Specific Function (R)-3-hydroxybutanoate + NAD(+) = acetoacetate + NADH
Enzyme 2 Pathways
Enzyme 2 Reactions
  • (R)-3-hydroxybutanoate + NAD+ = acetoacetate + NADH + H+ [RN:R01361] ALL_REAC R01361
Enzyme 2 Pfam Domain Function
Enzyme 2 Signals
  • None
Enzyme 2 Transmembrane Regions
  • None
Enzyme 2 Essentiality Not Available
Enzyme 2 GenBank ID Protein 8895083 Link Image
Enzyme 2 UniProtKB/Swiss-Prot ID Q9BUT1 Link Image
Enzyme 2 UniProtKB/Swiss-Prot Entry Name BDH2_HUMAN Link Image
Enzyme 2 PDB ID Not Available
Enzyme 2 Cellular Location Not Available
Enzyme 2 Gene Sequence Not Available
Enzyme 2 GenBank Gene ID AF164790 Link Image
Enzyme 2 GeneCard ID Not Available
Enzyme 2 GenAtlas ID BDH2 Link Image
Enzyme 2 HGNC ID HGNC:32389 Link Image
Enzyme 2 Chromosome Location Not Available
Enzyme 2 Locus Not Available
Enzyme 2 SNPs SNPJam Report Link Image
Enzyme 2 General References
  1. Hu RM, Han ZG, Song HD, Peng YD, Huang QH, Ren SX, Gu YJ, Huang CH, Li YB, Jiang CL, Fu G, Zhang QH, Gu BW, Dai M, Mao YF, Gao GF, Rong R, Ye M, Zhou J, Xu SH, Gu J, Shi JX, Jin WR, Zhang CK, Wu TM, Huang GY, Chen Z, Chen MD, Chen JL: Gene expression profiling in the human hypothalamus-pituitary-adrenal axis and full-length cDNA cloning. Proc Natl Acad Sci U S A. 2000 Aug 15;97(17):9543-8. [PubMed Link Image]
  2. Clark HF, Gurney AL, Abaya E, Baker K, Baldwin D, Brush J, Chen J, Chow B, Chui C, Crowley C, Currell B, Deuel B, Dowd P, Eaton D, Foster J, Grimaldi C, Gu Q, Hass PE, Heldens S, Huang A, Kim HS, Klimowski L, Jin Y, Johnson S, Lee J, Lewis L, Liao D, Mark M, Robbie E, Sanchez C, Schoenfeld J, Seshagiri S, Simmons L, Singh J, Smith V, Stinson J, Vagts A, Vandlen R, Watanabe C, Wieand D, Woods K, Xie MH, Yansura D, Yi S, Yu G, Yuan J, Zhang M, Zhang Z, Goddard A, Wood WI, Godowski P, Gray A: The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment. Genome Res. 2003 Oct;13(10):2265-70. Epub 2003 Sep 15. [PubMed Link Image]
Enzyme 2 Metabolite References Not Available