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Human Metabolome Database Version 3.5

Showing metabocard for Beta-Alanine (HMDB00056)

• Identification
• Taxonomy
• Ontology
• Physical properties
• Spectra
• Biological properties
• Concentrations
• Links
• References

enzymes (12) transporters (1)

• Blood
• Urine

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	Download: MOL | SDF | SMILES | InChI Display: 2D Structure | 3D Structure
Synonyms	2-Carboxyethylamine 3-Amino-Propanoate 3-Amino-Propanoic acid 3-Aminopropanoate 3-Aminopropanoic acid 3-Aminopropionate 3-Aminopropionic acid Abufene B-Alanine b-Aminopropanoate b-Aminopropanoic acid b-Aminopropionate b-Aminopropionic acid beta Alanine beta-Alanine beta-Aminopropanoate beta-Aminopropanoic acid beta-Aminopropionate beta-Aminopropionic acid Omega-Aminopropionate 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 Aspartate Metabolism SMP00067 map00250 Beta-Alanine Metabolism SMP00007 map00410 Pyrimidine Metabolism SMP00046 map00240
Normal Concentrations
	Biofluid Status Value Age Sex Condition Comments Blood Detected and Quantified 3.0 (1.3-20.0) uM Adult (>18 years old) Both Normal Not Available Blood Detected and Quantified 3.8 +/- 2.9 uM Adult (>18 years old) Both Normal Not Available Blood Detected and Quantified 3.00 +/- 0.85 uM Adult (>18 years old) Male Normal Not Available Blood Detected and Quantified 1.76 +/- 0.74 uM Adult (>18 years old) Female Normal Not Available Blood Detected and Quantified 3.0 +/- 0.85 uM Adult (>18 years old) Male Normal Not Available Blood Detected and Quantified 1.76 +/- 0.74 uM Adult (>18 years old) Female Normal Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 0.024 +/- 0.013 uM Adult (>18 years old) Both Normal Not Available Urine Detected and Quantified 2.4 +/- 3.0 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available Urine Detected and not Quantified Not Applicable Adult (>18 years old) Both Normal Not Available Urine Detected and Quantified 5.9 (3.4-13.0) umol/mmol creatinine Adult (>18 years old) Both Normal urine by NMR Urine Detected and not Quantified Not Applicable Adult (>18 years old) Both Normal Urine compound detected by GC-MS Urine Detected and Quantified 1.27 +/- 0.9 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available Urine Detected and Quantified 0.8 umol/mmol creatinine Adult (>18 years old) Both Normal Not Available Urine Detected and Quantified 0.23 +/- 0.73 umol/mmol creatinine Infant (0-1 year old) Both Normal Not Available Urine Detected and Quantified 0.00068 +/- 0.00049 umol/mmol creatinine Adult (>18 years old) Male Normal Not Available Urine Detected and Quantified 0.0007 +/- 0.00075 umol/mmol creatinine Adult (>18 years old) Female Normal Not Available
Abnormal Concentrations
	Biofluid Status Value Age Sex Condition Comments Blood Detected and Quantified 4.0 (0.9-6.2) uM Adult (>18 years old) Both Dihydropyrimidine dehydrogenase (DPD) deficiency Not Available Blood Detected and Quantified 2.7 +/- 1.3 uM Adult (>18 years old) Both Dihydropyrimidine dehydrogenase (DPD) deficiency Not Available Blood Detected and Quantified 23.0 uM Adult (>18 years old) Both GABA transaminase deficiency Not Available Blood Detected and Quantified 5.00 (0.00-10.00) uM Adult (>18 years old) Both Hyper beta-alaninemia Not Available Blood Detected and Quantified 107.5 (15.00-200.00) uM Adult (>18 years old) Both Hyper- beta-Alaninemia Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 0.036 +/- 0.034 uM Adult (>18 years old) Both Dihydropyrimidine dehydrogenase (DPD) deficiency Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 0.03 (0.00-0.06) uM Adult (>18 years old) Both GABA transaminase deficiency Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 0.48 uM Adult (>18 years old) Both GABA transaminase deficiency Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 0.03 (0.00-0.06) uM Adult (>18 years old) Both Hyper beta-alaninemia Not Available Cerebrospinal Fluid (CSF) Detected and Quantified 22.5 (0.00-45.00) uM Adult (>18 years old) Both Hyper- beta-Alaninemia Not Available Urine Detected and Quantified 1.4 +/- 1.4 umol/mmol creatinine Adult (>18 years old) Both Dihydropyrimidine dehydrogenase (DPD) deficiency Not Available Urine Detected and Quantified 0.0-2.3 umol/mmol creatinine Adult (>18 years old) Both GABA transaminase deficiency Not Available
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 Gaba-transaminase deficiency http://www.metagene.de/program/d.prg?mp=GABA%20TRANSAMINASE%20DEFICIENCY Hyper beta-alaninemia http://www.metagene.de/program/d.prg?mp=HYPER%20BETA-ALANINEMIA
Associated OMIM IDs	274270 (Dihydropyrimidine dehydrogenase deficiency) 237400 (Hyper beta-alaninemia) 613163 (Gaba-transaminase deficiency)
External Links
DrugBank ID	DB03107
Phenol Explorer Compound ID	Not Available
Phenol Explorer Metabolite ID	Not Available
FoodDB ID	FDB002253
KNApSAcK ID	C00001333
Chemspider ID	234
KEGG Compound ID	C00099
BioCyc ID	B-ALANINE
BiGG ID	33848
Wikipedia Link	beta-Alanine
NuGOwiki Link	HMDB00056
Metagene Link	HMDB00056
METLIN ID	5119
PubChem Compound	239
PDB ID	BAL
ChEBI ID	16958
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	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 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 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 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 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 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 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 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 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 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 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 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 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 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 Karmanskii IM: [Effect of pepsin on low density serum lipoproteins] Vopr Med Khim. 1977 Jul-Aug;23(4):530-4. Pubmed: 200005 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 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

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Enzymes
Name: 4-aminobutyrate aminotransferase, mitochondrial Reactions: (S)-3-amino-2-methylpropanoate + 2-oxoglutarate = 2-methyl-3-oxopropanoate + L-glutamate Gene Name: ABAT Uniprot ID: P80404 Protein Sequence: FASTA Gene Sequence: FASTA
Name: 4-trimethylaminobutyraldehyde dehydrogenase Reactions: 4-trimethylammoniobutanal + NAD+ + H2O = 4-trimethylammoniobutanoate + NADH + 2 H+ [RN:R03283] Gene Name: ALDH9A1 Uniprot ID: P49189 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Alpha-aminoadipic semialdehyde dehydrogenase Reactions: L-2-aminoadipate 6-semialdehyde + NAD(P)+ + H2O = L-2-aminoadipate + NAD(P)H + H+ [RN:R03102 R03103] Gene Name: ALDH7A1 Uniprot ID: P49419 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Aldehyde dehydrogenase family 1 member A3 Reactions: an aldehyde + NAD(P)+ + H2O = an acid + NAD(P)H + H+ [RN:R00538 R00634] Gene Name: ALDH1A3 Uniprot ID: P47895 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Aldehyde dehydrogenase, mitochondrial Reactions: an aldehyde + NAD+ + H2O = an acid + NADH + H+ [RN:R00538] Gene Name: ALDH2 Uniprot ID: P05091 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Fatty aldehyde dehydrogenase Reactions: an aldehyde + NAD+ + H2O = an acid + NADH + H+ [RN:R00538] Gene Name: ALDH3A2 Uniprot ID: P51648 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Aldehyde dehydrogenase X, mitochondrial Reactions: an aldehyde + NAD+ + H2O = an acid + NADH + H+ [RN:R00538] Gene Name: ALDH1B1 Uniprot ID: P30837 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Beta-Ala-His dipeptidase Reactions: Preferential hydrolysis of the beta-Ala!His dipeptide (carnosine), and also anserine, Xaa!His dipeptides and other dipeptides including homocarnosine [RN:R01166 R03288] ALL_REAC R01166 R03288 COFACTOR Citrate [CPD:C00158] Cadmium [CPD:C01413] Gene Name: CNDP1 Uniprot ID: Q96KN2 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Beta-ureidopropionase Reactions: N-carbamoyl-beta-alanine + H2O = beta-alanine + CO2 + NH3 [RN:R00905] Gene Name: UPB1 Uniprot ID: Q9UBR1 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glutamate decarboxylase 2 Reactions: L-glutamate = 4-aminobutanoate + CO2 [RN:R00261] Gene Name: GAD2 Uniprot ID: Q05329 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Glutamate decarboxylase 1 Reactions: L-glutamate = 4-aminobutanoate + CO2 [RN:R00261] Gene Name: GAD1 Uniprot ID: Q99259 Protein Sequence: FASTA Gene Sequence: FASTA
Name: Sodium- and chloride-dependent taurine transporter Reactions: --- [] Gene Name: SLC6A6 Uniprot ID: P31641 Protein Sequence: FASTA Gene Sequence: FASTA

Transporters
Name: Monocarboxylate transporter 10 Reactions: Gene Name: SLC16A10 Uniprot ID: Q8TF71 Protein Sequence: FASTA Gene Sequence: FASTA

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