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Record Information
StatusDetected and Quantified
Creation Date2006-08-13 07:07:14 UTC
Update Date2019-04-01 19:20:16 UTC
Secondary Accession Numbers
  • HMDB0000485
  • HMDB00485
  • HMDB03911
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, the last step in pyrimidine degradation. Beta-ureidopropionase deficiency is an inborn error of pyrimidine degradation associated with neurological abnormalities (OMIM 606673 ).
2-(Aminomethyl)propionic acidChEBI
3-Aminoisobutanoic acidChEBI
beta-Aminoisobutyric acidChEBI
DL-beta-Aminoisobutyric acidChEBI
3-Amino-2-methylpropanoic acidGenerator
b-Aminoisobutyric acidGenerator
Β-aminoisobutyric acidGenerator
DL-b-Aminoisobutyric acidGenerator
DL-Β-aminoisobutyric acidGenerator
3-Aminoisobutyric acidGenerator
(+/-)-3-amino-2-methylpropanoic acidHMDB
(+/-)-3-aminoisobutyric acidHMDB
(+/-)-b-aminoisobutyric acidHMDB
(+/-)-beta-aminoisobutyric acidHMDB
3-Amino-isobutanoic acidHMDB
DL-3-Amino-2-methylpropionic acidHMDB
DL-3-Aminoisobutyric acidHMDB
3-Aminoisobutyric acid, (+-)-isomerHMDB
DL-3-Aminoisobutyric acid monohydrateHMDB
3-Aminoisobutyric acid, (S)-isomerHMDB
3-Aminoisobutyric acid, tritium-labeledHMDB
3-Aminoisobutyric acid, (R)-isomerHMDB
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
InChI Identifier
Chemical Taxonomy
Description 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
  • Beta amino acid or derivatives
  • Amino acid
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Amine
  • Organic oxide
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Organopnictogen compound
  • Primary aliphatic amine
  • Organic oxygen compound
  • Carbonyl group
  • Organic nitrogen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors


Biological location:


Naturally occurring process:


Biological role:

Physical Properties
Experimental Properties
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
Water Solubility367 g/LALOGPS
pKa (Strongest Acidic)4.17ChemAxon
pKa (Strongest Basic)10.32ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area63.32 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity25.28 m³·mol⁻¹ChemAxon
Polarizability10.42 ųChemAxon
Number of Rings0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectrum TypeDescriptionSplash KeyView
GC-MSGC-MS Spectrum - GC-MS (1 TMS)splash10-001i-3900000000-9d996f1ef303093bc6f2JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-0udi-1900000000-599e217ab2fffbcaedcfJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-MS (3 TMS)splash10-00di-2920000000-235394017181e4a1ccc4JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00di-2920000000-235394017181e4a1ccc4JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-001i-3900000000-9d996f1ef303093bc6f2JSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-0udi-1900000000-599e217ab2fffbcaedcfJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00dr-2900000000-83730a256456aac8055aJSpectraViewer | MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-0udi-0900000000-ecf6ec14beb319448103JSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-001i-9000000000-89dae4e73d997d89e2e6JSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00ar-9200000000-0eb92539979a9fa36dbdJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000i-9200000000-1212facd6e8f1c4ede25JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0udi-6900000000-8b7d740c75c426976897JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0udi-4900000000-68b30ab5196763854bfbJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Positivesplash10-0udr-5900000000-d8bec01eb9aa047f87d0JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negativesplash10-0udi-2900000000-d13f4bc606699ca79bdaJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , negativesplash10-0udi-2900000000-d13f4bc606699ca79bdaJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - , negativesplash10-0uk9-5900000000-7eb5bc6532d9dac5e48fJSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QTOF , positivesplash10-0udr-5900000000-d8bec01eb9aa047f87d0JSpectraViewer | MoNA
LC-MS/MSLC-MS/MS Spectrum - , positivesplash10-0udr-9600000000-62b406ac77af506e283fJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0f79-9400000000-e80e2eb321cf19f4e299JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-000f-9000000000-9649dec1f6afdd5b12c6JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9000000000-aaf9fc6ded63400c1d6dJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0udi-2900000000-6f58da61e2ae030dbbadJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0zfr-9600000000-ba32a8e8fca536c36023JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0a4l-9000000000-e756dcf352426858cb71JSpectraViewer | MoNA
1D NMR1H NMR SpectrumNot AvailableJSpectraViewer
1D NMR1H NMR SpectrumNot AvailableJSpectraViewer
1D NMR13C NMR SpectrumNot AvailableJSpectraViewer
2D NMR[1H,13C] 2D NMR SpectrumNot AvailableJSpectraViewer
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Feces
  • Urine
Tissue LocationsNot Available
Normal Concentrations
BloodDetected and Quantified1.8-4.5 uMNewborn (0-30 days old)Not SpecifiedNormal details
BloodDetected and Quantified1.03 +/- 0.34 uMAdult (>18 years old)MaleNormal details
BloodDetected and Quantified0.75 +/- 0.33 uMAdult (>18 years old)FemaleNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
FecesDetected but not Quantified Not SpecifiedBoth
FecesDetected but not Quantified Adult (>18 years old)Both
UrineDetected and Quantified3.6-13 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified5.5 umol/mmol creatinineAdult (>18 years old)BothNormal details
UrineDetected and Quantified9.73 +/- 9.20 umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Geigy Scientific ...
UrineDetected and Quantified7.20 +/- 6.73 umol/mmol creatinineAdult (>18 years old)FemaleNormal
    • Geigy Scientific ...
UrineDetected and Quantified34.288 +/- 32.202 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
    • Mordechai, Hien, ...
UrineDetected and Quantified2.91-116.43 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
UrineDetected but not Quantified Adult (>18 years old)Both
UrineDetected and Quantified4.3-8.5 umol/mmol creatinineAdult (>18 years old)MaleNormal 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
UrineDetected and Quantified25.9 (2.2-140.0) umol/mmol creatinineAdult (>18 years old)Both
Abnormal Concentrations
BloodDetected and Quantified30 +/- 6 uMNewborn (0-30 days old)MaleMethylmalonate semialdehyde dehydrogenase deficiency details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
UrineDetected but not Quantified Adult (>18 years old)BothBladder cancer details
UrineDetected and Quantified60.338 +/- 80.333 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Mordechai, Hien, ...
UrineDetected and Quantified248.732 umol/mmol creatinineChildren (1-13 years old)MaleD-Lactic Acidosis details
Associated Disorders and Diseases
Disease References
Methylmalonate semialdehyde dehydrogenase deficiency
  1. Pollitt RJ, Green A, Smith R: Excessive excretion of beta-alanine and of 3-hydroxypropionic, R- and S-3-aminoisobutyric, R- and S-3-hydroxyisobutyric and S-2-(hydroxymethyl)butyric acids probably due to a defect in the metabolism of the corresponding malonic semialdehydes. J Inherit Metab Dis. 1985;8(2):75-9. [PubMed:3939535 ]
Colorectal cancer
  1. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
  2. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Eosinophilic esophagitis
  1. (). Mordechai, Hien, and David S. Wishart. .
D-Lactic Acidosis
  1. Duran M, Van Biervliet JP, Kamerling JP, Wadman SK: D-lactic aciduria, an inborn error of metabolism? Clin Chim Acta. 1977 Feb 1;74(3):297-300. [PubMed:832430 ]
Associated OMIM IDs
  • 614105 (Methylmalonate semialdehyde dehydrogenase deficiency)
  • 114500 (Colorectal cancer)
  • 610247 (Eosinophilic esophagitis)
  • 245450 (D-Lactic Acidosis)
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB023248
KNApSAcK IDNot Available
Chemspider ID58481
KEGG Compound IDC05145
BiGG ID41519
Wikipedia Link3-Aminoisobutyric_acid
PubChem Compound64956
PDB IDNot Available
ChEBI ID27389
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
  1. 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 ]
  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. [PubMed:15831902 ]
  3. Lee SH, Nam SY, Chung BC: Altered profile of endogenous steroids in the urine of patients with prolactinoma. Clin Biochem. 1998 Oct;31(7):529-35. [PubMed:9812172 ]
  4. 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:2026685 ]
  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. [PubMed:679461 ]
  6. Jin LJ, Wang Y, Xu R, Go ML, Lee HK, Li SF: Chiral resolution of atropine, homatropine and eight synthetic tropinyl and piperidinyl esters by capillary zone electrophoresis with cyclodextrin additives. Electrophoresis. 1999 Jan;20(1):198-203. [PubMed:10065977 ]
  7. LeBlanc J: Nutritional implications of cephalic phase thermogenic responses. Appetite. 2000 Apr;34(2):214-6. [PubMed:10744912 ]
  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. [PubMed: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. [PubMed:12119452 ]
  10. Joyner MJ, Dietz NM: Sympathetic vasodilation in human muscle. Acta Physiol Scand. 2003 Mar;177(3):329-36. [PubMed:12609003 ]
  11. Nelson CP, Gupta P, Napier CM, Nahorski SR, Challiss RA: Functional selectivity of muscarinic receptor antagonists for inhibition of M3-mediated phosphoinositide responses in guinea pig urinary bladder and submandibular salivary gland. J Pharmacol Exp Ther. 2004 Sep;310(3):1255-65. Epub 2004 May 12. [PubMed:15140916 ]
  12. Jooste EH, Sharma A, Zhang Y, Emala CW: Rapacuronium augments acetylcholine-induced bronchoconstriction via positive allosteric interactions at the M3 muscarinic receptor. Anesthesiology. 2005 Dec;103(6):1195-203. [PubMed:16306732 ]
  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. [PubMed: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. [PubMed: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. [PubMed:16730119 ]
  16. Hammer RH, Wu W, Sastry JS, Bodor N: Short acting soft mydriatics. Curr Eye Res. 1991 Jun;10(6):565-70. [PubMed:1893772 ]
  17. Salminen L: Review: systemic absorption of topically applied ocular drugs in humans. J Ocul Pharmacol. 1990 Fall;6(3):243-9. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed: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. [PubMed:7324829 ]
  29. Virtanen R, Kanto J, Iisalo E: Radioimmunoassay for atropine and l-hyoscyamine. Acta Pharmacol Toxicol (Copenh). 1980 Sep;47(3):208-12. [PubMed: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. [PubMed:7582538 ]
  31. Hirano H, Shimosegawa T, Meguro T, Shiga N, Koizumi M, Toyota T: Effects of ethanol on meal-stimulated secretion of pancreatic polypeptide and cholecystokinin: comparison of healthy volunteers, heavy drinkers, and patients with chronic pancreatitis. J Gastroenterol. 1996 Feb;31(1):86-93. [PubMed:8808434 ]
  32. Foster WM, Bergofsky EH, Bohning DE, Lippmann M, Albert RE: Effect of adrenergic agents and their mode of action on mucociliary clearance in man. J Appl Physiol. 1976 Aug;41(2):146-52. [PubMed:956095 ]
  33. Luik AI, Naboka YuN, Mogilevich SE, Hushcha TO, Mischenko NI: Study of human serum albumin structure by dynamic light scattering: two types of reactions under different pH and interaction with physiologically active compounds. Spectrochim Acta A Mol Biomol Spectrosc. 1998 Sep;54A(10):1503-7. [PubMed:9807240 ]
  34. Chaturvedi AK, Vu NT, Ritter RM, Canfield DV: DNA typing as a strategy for resolving issues relevant to forensic toxicology. J Forensic Sci. 1999 Jan;44(1):189-92. [PubMed:9987885 ]


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:
Uniprot ID:
Molecular weight:
Ureidoisobutyric acid + Water → 3-Aminoisobutanoic acid + Carbon dioxide + Ammoniadetails