Human Metabolome Database Version 3.5

Showing metabocard for Beta-Alanine (HMDB00056)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-02-08 17:07:49 -0700
HMDB ID HMDB00056
Secondary Accession Numbers None
Metabolite Identification
Common Name Beta-Alanine
Description Beta-alanine is the only naturally occurring beta-amino acid - the amino group is at the β-position from the carboxylate group. It is formed in vivo by the degradation of dihydrouracil and carnosine. It is a component of the naturally occurring peptides carnosine and anserine and also of pantothenic acid (Vitamin B-5) which itself is a component of coenzyme A. Under normal conditions, beta-alanine is metabolized into acetic acid. Since neuronal uptake and neuronal receptor sensitivity to beta-alanine have been demonstrated, the compound may be a false transmitter replacing gamma-aminobutyric acid. A rare genetic disorder, hyper-beta-alaninemia, has been reported.
Structure Thumb
Download: MOL | SDF | PDB | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. 2-Carboxyethylamine
  2. 3-Amino-Propanoate
  3. 3-Amino-Propanoic acid
  4. 3-Aminopropanoate
  5. 3-Aminopropanoic acid
  6. 3-Aminopropionate
  7. 3-Aminopropionic acid
  8. Abufene
  9. B-Alanine
  10. b-Aminopropanoate
  11. b-Aminopropanoic acid
  12. b-Aminopropionate
  13. b-Aminopropionic acid
  14. beta Alanine
  15. beta-Alanine
  16. beta-Aminopropanoate
  17. beta-Aminopropanoic acid
  18. beta-Aminopropionate
  19. beta-Aminopropionic acid
  20. Omega-Aminopropionate
  21. Omega-Aminopropionic acid
Chemical Formula C3H7NO2
Average Molecular Weight 89.0932
Monoisotopic Molecular Weight 89.047678473
IUPAC Name 3-aminopropanoic acid
Traditional IUPAC Name β-alanine
CAS Registry Number 107-95-9
SMILES NCCC(O)=O
InChI Identifier InChI=1S/C3H7NO2/c4-2-1-3(5)6/h1-2,4H2,(H,5,6)
InChI Key UCMIRNVEIXFBKS-UHFFFAOYSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Amino Acids, Peptides, and Analogues
Class Amino Acids and Derivatives
Sub Class Beta Amino Acids and Derivatives
Other Descriptors
  • Aliphatic Acyclic Compounds
  • Biogenic amines(KEGG)
  • Other amino acids(KEGG)
  • amino acid zwitterion(ChEBI)
Substituents
  • 1,3 Aminoalcohol
  • Carboxylic Acid
  • Primary Aliphatic Amine (Alkylamine)
Direct Parent Beta Amino Acids and Derivatives
Ontology
Status Detected and Quantified
Origin
  • Endogenous
Biofunction
  • Component of Pantothenate and CoA biosynthesis
  • Component of Pyrimidine metabolism
  • Component of beta-Alanine metabolism
Application Not Available
Cellular locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point 200 °C Not Available
Boiling Point Not Available Not Available
Water Solubility 545.0 mg/mL Not Available
LogP -3.05 TSAI,RS ET AL. (1991)
Predicted Properties
Property Value Source
Water Solubility 494 g/L ALOGPS
LogP -3.26 ALOGPS
LogP -3.2 ChemAxon
LogS 0.74 ALOGPS
pKa (strongest acidic) 4.08 ChemAxon
pKa (strongest basic) 10.31 ChemAxon
Hydrogen Acceptor Count 3 ChemAxon
Hydrogen Donor Count 2 ChemAxon
Polar Surface Area 63.32 A2 ChemAxon
Rotatable Bond Count 2 ChemAxon
Refractivity 20.7 ChemAxon
Polarizability 8.62 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge 0 ChemAxon
Spectra
Gas-MS Spectrum
1H NMR Spectrum
13C 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) 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
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies )
[1H,1H] 2D NMR Spectrum
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Mitochondria
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location
  • Muscle
  • Pancreas
  • Placenta
  • Kidney
  • Prostate
Pathways
Name SMPDB Link KEGG Link
Propanoate Metabolism SMP00016 map00640 Link_out
Aspartate Metabolism SMP00067 map00250 Link_out
Beta-Alanine Metabolism SMP00007 map00410 Link_out
Pyrimidine Metabolism SMP00046 map00240 Link_out
Normal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified
3.0 (1.3-20.0) uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
3.8 +/- 2.9 uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
5.00 (0.00-10.00) uM Adult (>18 years old) Both Normal
Blood Detected and Quantified
3.00 +/- 0.85 uM Adult (>18 years old) Male Normal
  • Geigy Scient...
Blood Detected and Quantified
1.76 +/- 0.74 uM Adult (>18 years old) Female Normal
  • Geigy Scient...
Blood Detected and Quantified
3.0 +/- 0.85 uM Adult (>18 years old) Male Normal
Blood Detected and Quantified
1.76 +/- 0.74 uM Adult (>18 years old) Female Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
0.024 +/- 0.013 uM Adult (>18 years old) Both Normal
Cerebrospinal Fluid (CSF) Detected and Quantified
0.03 (0.00-0.06) uM Adult (>18 years old) Both Normal
Urine Detected and Quantified
2.4 +/- 3.0 umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Both Normal
Urine Detected and Quantified
5.9 (3.4-13.0) umol/mmol creatinine Adult (>18 years old) Both Comment Normal
Urine Detected but not Quantified
Not Applicable Adult (>18 years old) Both Comment Normal
Urine Detected and Quantified
1.27 +/- 0.9 umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
0.8 umol/mmol creatinine Adult (>18 years old) Both Normal
Urine Detected and Quantified
0.23 +/- 0.73 umol/mmol creatinine Infant (0-1 year old) Both Normal
Urine Detected and Quantified
0.00068 +/- 0.00049 umol/mmol creatinine Adult (>18 years old) Male Normal
Urine Detected and Quantified
0.0007 +/- 0.00075 umol/mmol creatinine Adult (>18 years old) Female Normal
Abnormal Concentrations
Biofluid Status Value Age Sex Condition Reference
Blood Detected and Quantified 4.0 (0.9-6.2) uM Adult (>18 years old) Both Dihydropyrimidine dehydrogenase (DPD) deficiency
Blood Detected and Quantified 2.7 +/- 1.3 uM Adult (>18 years old) Both Dihydropyrimidine dehydrogenase (DPD) deficiency
Blood Detected and Quantified 23.0 uM Children (1-13 year old) Both GABA transaminase deficiency
Blood Detected and Quantified 107.5 (15.00-200.00) uM Children (1-13 year old) Both Hyper- beta-Alaninemia
Cerebrospinal Fluid (CSF) Detected and Quantified 0.036 +/- 0.034 uM Adult (>18 years old) Both Dihydropyrimidine dehydrogenase (DPD) deficiency
Cerebrospinal Fluid (CSF) Detected and Quantified 0.48 uM Children (1-13 year old) Both GABA transaminase deficiency
Cerebrospinal Fluid (CSF) Detected and Quantified 22.5 (0.00-45.00) uM Children (1-13 year old) Both Hyper- beta-Alaninemia
Urine Detected and Quantified 1.4 +/- 1.4 umol/mmol creatinine Adult (>18 years old) Both Dihydropyrimidine dehydrogenase (DPD) deficiency
Urine Detected and Quantified 0.0-2.3 umol/mmol creatinine Children (1-13 year old) Both GABA transaminase deficiency
Associated Disorders and Diseases
Disease References
Dihydropyrimidine dehydrogenase deficiency
  • Van Kuilenburg AB, Stroomer AE, Van Lenthe H, Abeling NG, Van Gennip AH: New insights in dihydropyrimidine dehydrogenase deficiency: a pivotal role for beta-aminoisobutyric acid? Biochem J. 2004 Apr 1;379(Pt 1):119-24. Pubmed: 14705962 Link_out
      Gaba-transaminase deficiency
          Hyper beta-alaninemia
              Associated OMIM IDs
              • 274270 Link_out (Dihydropyrimidine dehydrogenase deficiency)
              • 613163 Link_out (Gaba-transaminase deficiency)
              • 237400 Link_out (Hyper beta-alaninemia)
              DrugBank ID DB03107 Link_out
              DrugBank Metabolite ID Not Available
              Phenol Explorer Compound ID Not Available
              Phenol Explorer Metabolite ID Not Available
              FoodDB ID FDB002253
              KNApSAcK ID C00001333 Link_out
              Chemspider ID 234 Link_out
              KEGG Compound ID C00099 Link_out
              BioCyc ID B-ALANINE Link_out
              BiGG ID 33848 Link_out
              Wikipedia Link beta-Alanine Link_out
              NuGOwiki Link HMDB00056 Link_out
              Metagene Link HMDB00056 Link_out
              METLIN ID 5119 Link_out
              PubChem Compound 239 Link_out
              PDB ID BAL Link_out
              ChEBI ID 16958 Link_out
              References
              Synthesis Reference Buc, Saul R.; Ford, Jared H.; Wise, E. C. Improved synthesis of b-alanine. Journal of the American Chemical Society (1945), 67 92-4.
              Material Safety Data Sheet (MSDS) Download (PDF)
              General References
              1. Van Kuilenburg AB, Stroomer AE, Van Lenthe H, Abeling NG, Van Gennip AH: New insights in dihydropyrimidine dehydrogenase deficiency: a pivotal role for beta-aminoisobutyric acid? Biochem J. 2004 Apr 1;379(Pt 1):119-24. Pubmed: 14705962 Link_out
              2. Malet-Martino MC, Bernadou J, Martino R, Armand JP: 19F NMR spectrometry evidence for bile acid conjugates of alpha-fluoro-beta-alanine as the main biliary metabolites of antineoplastic fluoropyrimidines in humans. Drug Metab Dispos. 1988 Jan-Feb;16(1):78-84. Pubmed: 2894959 Link_out
              3. Klebanov GI, Teselkin YuO, Babenkova IV, Lyubitsky OB, Rebrova OYu, Boldyrev AA, Vladimirov YuA: Effect of carnosine and its components on free-radical reactions. Membr Cell Biol. 1998;12(1):89-99. Pubmed: 9829262 Link_out
              4. Aznar J, Gilabert J, Estelles A, Fernandez MA, Villa P, Aznar JA: Evaluation of the soluble fibrin monomer complexes and other coagulation parameters in obstetric patients. Thromb Res. 1982 Sep 15;27(6):691-701. Pubmed: 7179210 Link_out
              5. Champion EE, Mann SJ, Glazier JD, Jones CJ, Rawlings JM, Sibley CP, Greenwood SL: System beta and system A amino acid transporters in the feline endotheliochorial placenta. Am J Physiol Regul Integr Comp Physiol. 2004 Dec;287(6):R1369-79. Epub 2004 Jul 29. Pubmed: 15284084 Link_out
              6. Kuo KC, Cole TF, Gehrke CW, Waalkes TP, Borek E: Dual-column cation-exchange chromatographic method for beta-aminoisobutyric acid and beta-alanine in biological samples. Clin Chem. 1978 Aug;24(8):1373-80. Pubmed: 679461 Link_out
              7. van Kuilenburg AB, Meinsma R, Beke E, Assmann B, Ribes A, Lorente I, Busch R, Mayatepek E, Abeling NG, van Cruchten A, Stroomer AE, van Lenthe H, Zoetekouw L, Kulik W, Hoffmann GF, Voit T, Wevers RA, Rutsch F, van Gennip AH: beta-Ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities. Hum Mol Genet. 2004 Nov 15;13(22):2793-801. Epub 2004 Sep 22. Pubmed: 15385443 Link_out
              8. Heggie GD, Sommadossi JP, Cross DS, Huster WJ, Diasio RB: Clinical pharmacokinetics of 5-fluorouracil and its metabolites in plasma, urine, and bile. Cancer Res. 1987 Apr 15;47(8):2203-6. Pubmed: 3829006 Link_out
              9. Gibson KM, Schor DS, Gupta M, Guerand WS, Senephansiri H, Burlingame TG, Bartels H, Hogema BM, Bottiglieri T, Froestl W, Snead OC, Grompe M, Jakobs C: Focal neurometabolic alterations in mice deficient for succinate semialdehyde dehydrogenase. J Neurochem. 2002 Apr;81(1):71-9. Pubmed: 12067239 Link_out
              10. Holm B, Nilsen DW, Kierulf P, Godal HC: Purification and characterization of 3 fibrinogens with different molecular weights obtained from normal human plasma. Thromb Res. 1985 Jan 1;37(1):165-76. Pubmed: 3983897 Link_out
              11. Chen Y, Getchell TV, Sparks DL, Getchell ML: Cellular localization of carnosinase in the human nasal mucosa. Acta Otolaryngol. 1994 Mar;114(2):193-8. Pubmed: 8203202 Link_out
              12. Milasta S, Pediani J, Appelbe S, Trim S, Wyatt M, Cox P, Fidock M, Milligan G: Interactions between the Mas-related receptors MrgD and MrgE alter signalling and trafficking of MrgD. Mol Pharmacol. 2006 Feb;69(2):479-91. Epub 2005 Nov 9. Pubmed: 16282220 Link_out
              13. Harris RC, Tallon MJ, Dunnett M, Boobis L, Coakley J, Kim HJ, Fallowfield JL, Hill CA, Sale C, Wise JA: The absorption of orally supplied beta-alanine and its effect on muscle carnosine synthesis in human vastus lateralis. Amino Acids. 2006 May;30(3):279-89. Epub 2006 Mar 24. Pubmed: 16554972 Link_out
              14. Hibbard JU, Pridjian G, Whitington PF, Moawad AH: Taurine transport in the in vitro perfused human placenta. Pediatr Res. 1990 Jan;27(1):80-4. Pubmed: 2296474 Link_out
              15. Karmanskii IM: [Effect of pepsin on low density serum lipoproteins] Vopr Med Khim. 1977 Jul-Aug;23(4):530-4. Pubmed: 200005 Link_out
              16. Johnson MR, Barnes S, Sweeny DJ, Diasio RB: 2-Fluoro-beta-alanine, a previously unrecognized substrate for bile acid coenzyme A:amino acid:N-acyltransferase from human liver. Biochem Pharmacol. 1990 Sep 15;40(6):1241-6. Pubmed: 2119585 Link_out
              17. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. Pubmed: 19212411 Link_out

              Enzymes
              Name: 4-aminobutyrate aminotransferase, mitochondrial
              Reactions:
              Beta-Alanine + Oxoglutaric acid unknown Malonic semialdehyde + L-Glutamic acid details
              Gene Name: ABAT
              Uniprot ID: P80404 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: 4-trimethylaminobutyraldehyde dehydrogenase
              Reactions:
              3-Aminopropionaldehyde + NAD + Water unknown Beta-Alanine + NADH + Hydrogen Ion details
              Gene Name: ALDH9A1
              Uniprot ID: P49189 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Aldehyde dehydrogenase, dimeric NADP-preferring
              Reactions:
              3-Aminopropionaldehyde + NAD + Water unknown Beta-Alanine + NADH + Hydrogen Ion details
              Gene Name: ALDH3A1
              Uniprot ID: P30838 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Alpha-aminoadipic semialdehyde dehydrogenase
              Reactions:
              3-Aminopropionaldehyde + NAD + Water unknown Beta-Alanine + NADH + Hydrogen Ion details
              Gene Name: ALDH7A1
              Uniprot ID: P49419 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Aldehyde dehydrogenase family 1 member A3
              Reactions:
              3-Aminopropionaldehyde + NAD + Water unknown Beta-Alanine + NADH + Hydrogen Ion details
              Gene Name: ALDH1A3
              Uniprot ID: P47895 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Aldehyde dehydrogenase, mitochondrial
              Reactions:
              3-Aminopropionaldehyde + NAD + Water unknown Beta-Alanine + NADH + Hydrogen Ion details
              Gene Name: ALDH2
              Uniprot ID: P05091 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Fatty aldehyde dehydrogenase
              Reactions:
              3-Aminopropionaldehyde + NAD + Water unknown Beta-Alanine + NADH + Hydrogen Ion details
              Gene Name: ALDH3A2
              Uniprot ID: P51648 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Aldehyde dehydrogenase X, mitochondrial
              Reactions:
              3-Aminopropionaldehyde + NAD + Water unknown Beta-Alanine + NADH + Hydrogen Ion details
              Gene Name: ALDH1B1
              Uniprot ID: P30837 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Beta-Ala-His dipeptidase
              Reactions: Not Available
              Gene Name: CNDP1
              Uniprot ID: Q96KN2 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Aldehyde dehydrogenase family 3 member B2
              Reactions:
              3-Aminopropionaldehyde + NAD + Water unknown Beta-Alanine + NADH + Hydrogen Ion details
              Gene Name: ALDH3B2
              Uniprot ID: P48448 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Aldehyde dehydrogenase family 3 member B1
              Reactions:
              3-Aminopropionaldehyde + NAD + Water unknown Beta-Alanine + NADH + Hydrogen Ion details
              Gene Name: ALDH3B1
              Uniprot ID: P43353 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Beta-ureidopropionase
              Reactions:
              Ureidopropionic acid + Water unknown Beta-Alanine + CO(2) + Ammonia details
              Ureidopropionic acid + Water unknown Beta-Alanine + Carbon dioxide + Ammonia details
              Gene Name: UPB1
              Uniprot ID: Q9UBR1 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Glutamate decarboxylase 2
              Reactions:
              L-Aspartic acid unknown Beta-Alanine + Carbon dioxide details
              Gene Name: GAD2
              Uniprot ID: Q05329 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Glutamate decarboxylase 1
              Reactions:
              L-Aspartic acid unknown Beta-Alanine + Carbon dioxide details
              Gene Name: GAD1
              Uniprot ID: Q99259 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Sodium- and chloride-dependent taurine transporter
              Reactions: Not Available
              Gene Name: SLC6A6
              Uniprot ID: P31641 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Name: Carnosine synthase 1
              Reactions:
              Adenosine triphosphate + L-Histidine + Beta-Alanine unknown ADP + Phosphoric acid + Carnosine details
              Adenosine triphosphate + L-Lysine + Beta-Alanine unknown Adenosine monophosphate + Pyrophosphate + beta-Alanyl-L-lysine details
              Adenosine triphosphate + L-Arginine + Beta-Alanine unknown Adenosine monophosphate + Pyrophosphate + beta-Alanyl-L-arginine details
              Adenosine triphosphate + L-Histidine + Beta-Alanine unknown Adenosine monophosphate + Pyrophosphate + Carnosine details
              Adenosine triphosphate + 3-Methylhistidine + Beta-Alanine unknown Adenosine monophosphate + Pyrophosphate + Anserine details
              Gene Name: CARNS1
              Uniprot ID: A5YM72 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA
              Transporters
              Name: Monocarboxylate transporter 10
              Reactions: Not Available
              Gene Name: SLC16A10
              Uniprot ID: Q8TF71 Link_out
              Protein Sequence: FASTA
              Gene Sequence: FASTA