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Record Information
Version3.6
Creation Date2006-08-13 07:07:14 UTC
Update Date2016-02-11 01:06:28 UTC
HMDB IDHMDB03911
Secondary Accession Numbers
  • HMDB00485
Metabolite Identification
Common Name3-Aminoisobutanoic acid
Descriptionbeta-aminoisobutyric acid is the product from the conversion of N-carbamyl-beta-aminoisobutyric acid by the enzyme Beta-ureidopropionase (EC 3.5.1.6), the last step in pyrimidine degradation. Beta-ureidopropionase deficiency is an inborn error of pyrimidine degradation associated with neurological abnormalities (OMIM 606673 ).
Structure
Thumb
Synonyms
ValueSource
2-(Aminomethyl)propionic acidChEBI
2-Methyl-beta-alanineChEBI
3-amino-2-MethylpropanoateChEBI
3-AminoisobutanoateChEBI
3-Aminoisobutanoic acidChEBI
alpha-Methyl-beta-alanineChEBI
BAIBChEBI
beta-Aminoisobutyric acidChEBI
DL-beta-Aminoisobutyric acidChEBI
2-(Aminomethyl)propionateGenerator
2-Methyl-b-alanineGenerator
2-Methyl-β-alanineGenerator
a-Methyl-b-alanineGenerator
α-methyl-β-alanineGenerator
b-AminoisobutyrateGenerator
b-Aminoisobutyric acidGenerator
beta-AminoisobutyrateGenerator
β-aminoisobutyrateGenerator
β-aminoisobutyric acidGenerator
DL-b-AminoisobutyrateGenerator
DL-b-Aminoisobutyric acidGenerator
DL-beta-AminoisobutyrateGenerator
DL-β-aminoisobutyrateGenerator
DL-β-aminoisobutyric acidGenerator
(+/-)-3-amino-2-methylpropanoateHMDB
(+/-)-3-amino-2-methylpropanoic acidHMDB
(+/-)-3-aminoisobutyric acidHMDB
(+/-)-b-aminoisobutyric acidHMDB
(+/-)-beta-aminoisobutyric acidHMDB
3-amino-IsobutanoateHMDB
3-amino-Isobutanoic acidHMDB
3-AminoisobutyrateHMDB
3-Aminoisobutyric acidHMDB
DL-2-Methyl-b-alanineHMDB
DL-2-Methyl-beta-alanineHMDB
DL-3-amino-2-Methylpropionic acidHMDB
DL-3-Aminoisobutyric acidHMDB
Chemical FormulaC4H9NO2
Average Molecular Weight103.1198
Monoisotopic Molecular Weight103.063328537
IUPAC Name3-amino-2-methylpropanoic acid
Traditional Name(+)-β-aminoisobutyric acid
CAS Registry Number144-90-1
SMILES
CC(CN)C(O)=O
InChI Identifier
InChI=1S/C4H9NO2/c1-3(2-5)4(6)7/h3H,2,5H2,1H3,(H,6,7)
InChI KeyInChIKey=QCHPKSFMDHPSNR-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as beta amino acids and derivatives. These are amino acids having a (-NH2) group attached to the beta carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentBeta amino acids and derivatives
Alternative Parents
Substituents
  • Beta amino acid or derivatives
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Carbonyl group
  • Amine
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
Biofunction
  • Protein synthesis, amino acid biosynthesis
ApplicationNot Available
Cellular locations
  • Cytoplasm (predicted from logP)
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility367.0 mg/mLALOGPS
logP-3ALOGPS
logP-2.6ChemAxon
logS0.55ALOGPS
pKa (Strongest Acidic)4.17ChemAxon
pKa (Strongest Basic)10.32ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity25.28 m3·mol-1ChemAxon
Polarizability10.42 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-MS (1 TMS)splash10-001i-3900000000-9d996f1ef303093bc6f2View in MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-0udi-1900000000-599e217ab2fffbcaedcfView in MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-00di-2920000000-235394017181e4a1ccc4View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000i-9200000000-1212facd6e8f1c4ede25View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0udi-6900000000-8b7d740c75c426976897View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0udi-4900000000-68b30ab5196763854bfbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positivesplash10-0udr-5900000000-d8bec01eb9aa047f87d0View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-0udi-2900000000-d13f4bc606699ca79bdaView in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Beta Ureidopropionase DeficiencySMP00172Not Available
Dihydropyrimidinase DeficiencySMP00178Not Available
MNGIE (Mitochondrial Neurogastrointestinal Encephalopathy)SMP00202Not Available
Pyrimidine MetabolismSMP00046map00240
UMP Synthase Deiciency (Orotic Aciduria)SMP00219Not Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified1.03 +/- 0.34 uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified0.75 +/- 0.33 uMAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified5.5 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified0.0042 +/- 0.002 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified0.0058 +/- 0.0041 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified34.288 +/- 32.202 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Mordechai, Hien, ...
details
UrineDetected and Quantified2.91-116.43 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified6.81 +/- 2.82 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified25.8 (1.4-155.6) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified25.9 (2.2-140.0) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified60.338 +/- 80.333 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
details
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB023248
KNApSAcK IDNot Available
Chemspider ID58481
KEGG Compound IDC05145
BioCyc ID3-AMINO-ISOBUTYRATE
BiGG ID41519
Wikipedia LinkNot Available
NuGOwiki LinkHMDB03911
Metagene LinkHMDB03911
METLIN ID5472
PubChem Compound64956
PDB IDNot Available
ChEBI ID27389
References
Synthesis ReferenceBalenovic, K.; Jambresic, I.; Ranogajec, I. Preparation of b-aminoisobutyric acid from glycine via the Wolff rearrangement of diazoethyl ketones. Croatica Chemica Acta (1957), 29 87-92.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
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  2. Fornai M, Blandizzi C, Colucci R, Antonioli L, Bernardini N, Segnani C, Baragatti B, Barogi S, Berti P, Spisni R, Del Tacca M: Role of cyclooxygenases 1 and 2 in the modulation of neuromuscular functions in the distal colon of humans and mice. Gut. 2005 May;54(5):608-16. [15831902 ]
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  5. 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. [679461 ]
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  8. Takeda H, Yamazaki Y, Akahane M, Igawa Y, Ajisawa Y, Nishizawa O: Role of the beta(3)-adrenoceptor in urine storage in the rat: comparison between the selective beta(3)-adrenoceptor agonist, CL316, 243, and various smooth muscle relaxants. J Pharmacol Exp Ther. 2000 Jun;293(3):939-45. [10869395 ]
  9. Raufman JP, Chen Y, Zimniak P, Cheng K: Deoxycholic acid conjugates are muscarinic cholinergic receptor antagonists. Pharmacology. 2002 Aug;65(4):215-21. [12119452 ]
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  13. Morgan CJ, Friedmann PS, Church MK, Clough GF: Cutaneous microdialysis as a novel means of continuously stimulating eccrine sweat glands in vivo. J Invest Dermatol. 2006 Jun;126(6):1220-5. [16470172 ]
  14. Carnicella S, de Vasconcelos AP, Pain L, Majchrzak M, Oberling P: Fos immunolabelling evidence for brain regions involved in the Pavlovian degraded contingency effect and in its disruption by atropine. Neuropharmacology. 2006 Jul;51(1):102-11. Epub 2006 May 6. [16678862 ]
  15. Hu J, DeChon J, Yan KC, Liu Q, Hu G, Wu J: Iptakalim inhibits nicotinic acetylcholine receptor-mediated currents in dopamine neurons acutely dissociated from rat substantia nigra pars compacta. Neurosci Lett. 2006 Jul 31;403(1-2):57-62. Epub 2006 May 26. [16730119 ]
  16. Hammer RH, Wu W, Sastry JS, Bodor N: Short acting soft mydriatics. Curr Eye Res. 1991 Jun;10(6):565-70. [1893772 ]
  17. Salminen L: Review: systemic absorption of topically applied ocular drugs in humans. J Ocul Pharmacol. 1990 Fall;6(3):243-9. [2290070 ]
  18. Watson GE, Pearson SK, Falany JL, Culp DJ, Tabak LA, Bowen WH: The effect of chronic atropine treatment on salivary composition and caries in rats. J Dent Res. 1989 Dec;68(12):1739-45. [2600253 ]
  19. Foster WM, Bergofsky EH: Airway mucus membrane: effects of beta-adrenergic and anticholinergic stimulation. Am J Med. 1986 Nov 14;81(5A):28-35. [2878612 ]
  20. Kanto J, Scheinin M: Placental and blood-CSF transfer of orally administered diazepam in the same person. Pharmacol Toxicol. 1987 Jul;61(1):72-4. [3114732 ]
  21. Kanto J, Klotz U: Pharmacokinetic implications for the clinical use of atropine, scopolamine and glycopyrrolate. Acta Anaesthesiol Scand. 1988 Feb;32(2):69-78. [3279717 ]
  22. Kusunoki M, Yamamura T, Takahashi T, Kantoh M, Ishikawa Y, Utsunomiya J: Choledochal cyst. Its possible autonomic involvement in the bile duct. Arch Surg. 1987 Sep;122(9):997-1000. [3619630 ]
  23. Imamura I, Watanabe T, Sakamoto Y, Wakamiya T, Shiba T, Hase Y, Tsuruhara T, Wada H: N tau-Ribosylhistidine, a novel histidine derivative in urine of histidinemic patients. Isolation, structure, and tissue level. J Biol Chem. 1985 Sep 5;260(19):10528-30. [4030756 ]
  24. Thommesen P, Funch-Jensen P, Taagehoj Jensen F: The influence of atropine and adrenaline on basal lymph flow in the neck region. Lymphology. 1979 Dec;12(4):272-4. [529832 ]
  25. Sato K, Sato F: Defective beta adrenergic response of cystic fibrosis sweat glands in vivo and in vitro. J Clin Invest. 1984 Jun;73(6):1763-71. [6327771 ]
  26. van Hooff M, van Baak MA, Schols M, Rahn KH: Studies of salivary flow in borderline hypertension: effects of drugs acting on structures innervated by the autonomic nervous system. Clin Sci (Lond). 1984 May;66(5):599-604. [6705484 ]
  27. Kubo K, Oka M, Frey R: Decrease of basal plasma histamine levels by application of atropine in man. Anaesthesist. 1982 Apr;31(4):172-3. [7091635 ]
  28. Kanto J, Virtanen R, Iisalo E, Maenpaa K, Liukko P: Placental transfer and pharmacokinetics of atropine after a single maternal intravenous and intramuscular administration. Acta Anaesthesiol Scand. 1981 Apr;25(2):85-8. [7324829 ]
  29. Virtanen R, Kanto J, Iisalo E: Radioimmunoassay for atropine and l-hyoscyamine. Acta Pharmacol Toxicol (Copenh). 1980 Sep;47(3):208-12. [7446137 ]
  30. Andersson K, Arner P: Cholinoceptor-mediated effects on glycerol output from human adipose tissue using in situ microdialysis. Br J Pharmacol. 1995 Aug;115(7):1155-62. [7582538 ]
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Enzymes

General function:
Involved in hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds
Specific function:
Converts N-carbamyl-beta-aminoisobutyric acid and N-carbamyl-beta-alanine to, respectively, beta-aminoisobutyric acid and beta-alanine, ammonia and carbon dioxide.
Gene Name:
UPB1
Uniprot ID:
Q9UBR1
Molecular weight:
43165.705
Reactions
Ureidoisobutyric acid + Water → 3-Aminoisobutanoic acid + Carbon dioxide + Ammoniadetails