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Human Metabolome Database Version 2.5

 

Showing metabocard for 3-Aminoisobutanoic acid (HMDB03911)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2006-08-13 08:07:14
Update Date 2009-10-21 23:18:41
Accession Number HMDB03911
Secondary Accession Numbers HMDB00485
Common Name 3-Aminoisobutanoic acid
Description beta-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)
Synonyms
  1. (+/-)-3-Amino-2-methylpropanoic acid
  2. (+/-)-3-Aminoisobutyric acid
  3. (+/-)-b-Aminoisobutyric acid
  4. 2-Methyl-b-alanine
  5. 3-Aminoisobutanoate
  6. DL-2-Methyl-b-alanine
  7. DL-3-Amino-2-methylpropionic acid
  8. a-Methyl-b-alanine
  9. b-Aminoisobutyric acid
  10. (+/-)-beta-Aminoisobutyric acid
  11. DL-beta-Aminoisobutyric acid
  12. 2-Methyl-beta-alanine
  13. DL-2-Methyl-beta-alanine
  14. alpha-Methyl-beta-alanine
  15. beta-Aminoisobutyric acid
  16. (+/-)-3-Amino-2-methylpropanoate
  17. 3-Aminoisobutanoic acid
Chemical IUPAC Name 3-Amino-2-methylpropanoate
Chemical Formula C4H9NO2
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Amino acids and Amino Acid conjugates
Class
  • Amino Acids
Sub Class
  • NA
Family
  • Mammalian Metabolite
Species
  • primary amine
  • primary aliphatic amine (alkylamine)
  • carboxylic acid
Biofunction
  • Protein synthesis, amino acid biosynthesis
Application
Source
  • Exogenous
Average Molecular Weight 103.120
Monoisotopic Molecular Weight 103.063332
Isomeric SMILES CC(CN)C(O)=O
Canonical SMILES CC(CN)C(O)=O
KEGG Compound ID C05145 Link Image
BioCyc ID 3-AMINO-ISOBUTYRATE Link Image
BiGG ID 41519 Link Image
Wikipedia Link Not Available
NuGOwiki Link HMDB03911 Link Image
Metagene Link HMDB03911 Link Image
METLIN ID 5472 Link Image
PubChem Compound 64956 Link Image
PubChem Substance 7413401 Link Image
ChEBI ID 16684 Link Image
CAS Registry Number 144-90-1
InChI Identifier InChI=1/C4H9NO2/c1-3(2-5)4(6)7/h3H,2,5H2,1H3,(H,6,7)
Synthesis Reference Balenovic, 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.
Melting Point (Experimental) Not Available
Experimental Water Solubility Not Available Source: PhysProp
Predicted Water Solubility 367.0 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge 0
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity -2.97 [Predicted by ALOGPS]; -0.7 [Predicted by PubChem via XLOGP] 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 (Bruker)
Show Experimental Conditions Link Image
Experimental 13C NMR Spectrum Not Available
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 Not Available
BMRB Spectrum Not Available
Cellular Location
  • Cytoplasm (Predicted from LogP)
Biofluid Location
  • Blood
  • Urine
Tissue Location Not Available
Concentrations (Normal)
Biofluid Blood
Value 1.03 +/- 0.34 uM
Age Adult:>18 yrs old
Sex Male
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Biofluid Blood
Value 0.75 +/- 0.33 uM
Age Adult:>18 yrs old
Sex Female
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Biofluid Urine
Value 0.0042 +/- 0.002 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Male
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 0.0058 +/- 0.0041 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Female
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 0.0042 +/- 0.0019 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Male
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Biofluid Urine
Value 0.0058 +/- 0.0041 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Female
Patient information Normal
Comments Not Available
References
  • 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 Link Image]
Biofluid Urine
Value 5.5 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Guo K, Li L: Differential (12)C-/(13)C-Isotope Dansylation Labeling and Fast Liquid Chromatography/Mass Spectrometry for Absolute and Relative Quantification of the Metabolome. Anal Chem. 2009 Mar 23. [PubMed Link Image]
Concentrations (Abnormal) Not Available
Associated Disorders Not Available
OMIM ID Not Available
Pathways
Name SMPDB Link KEGG Link
Pyrimidine Metabolism SMP00046 Link Image map00240 Link Image
General References
  1. 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 Link Image]
  2. LeBlanc J: Nutritional implications of cephalic phase thermogenic responses. Appetite. 2000 Apr;34(2):214-6. [PubMed Link Image]
  3. 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 Link Image]
  4. Raufman JP, Chen Y, Zimniak P, Cheng K: Deoxycholic acid conjugates are muscarinic cholinergic receptor antagonists. Pharmacology. 2002 Aug;65(4):215-21. [PubMed Link Image]
  5. Joyner MJ, Dietz NM: Sympathetic vasodilation in human muscle. Acta Physiol Scand. 2003 Mar;177(3):329-36. [PubMed Link Image]
  6. 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 Link Image]
  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 Link Image]
  8. 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 Link Image]
  9. 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 Link Image]
  10. 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 Link Image]
  11. 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 Link Image]
  12. 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 Link Image]
  13. Hammer RH, Wu W, Sastry JS, Bodor N: Short acting soft mydriatics. Curr Eye Res. 1991 Jun;10(6):565-70. [PubMed Link Image]
  14. 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]
  15. Salminen L: Review: systemic absorption of topically applied ocular drugs in humans. J Ocul Pharmacol. 1990 Fall;6(3):243-9. [PubMed Link Image]
  16. 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 Link Image]
  17. 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 Link Image]
  18. 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 Link Image]
  19. 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 Link Image]
  20. 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 Link Image]
  21. 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 Link Image]
  22. 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 Link Image]
  23. 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 Link Image]
  24. 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 Link Image]
  25. 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 Link Image]
  26. 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 Link Image]
  27. 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 Link Image]
  28. Virtanen R, Kanto J, Iisalo E: Radioimmunoassay for atropine and l-hyoscyamine. Acta Pharmacol Toxicol (Copenh). 1980 Sep;47(3):208-12. [PubMed Link Image]
  29. 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 Link Image]
  30. 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 Link Image]
  31. 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 Link Image]
  32. 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 Link Image]
  33. 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 Link Image]
  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 Link Image]
Metabolic Enzymes
  1. UPB1 protein
Enzyme 1 [top]
Enzyme 1 ID 12999
Enzyme 1 Name UPB1 protein
Enzyme 1 Synonyms
  1. Ureidopropionase, beta, isoform CRA_b
Enzyme 1 Gene Name UPB1
Enzyme 1 Protein Sequence >UPB1 protein
MAGAEWKSLEECLEKHLPLPDLQEVKRVLYGKELRKLDLPREAFEAASREDFELQGYAFE
AAEEQLRRPRIVHVGLVQNRIPLPANAPVAEQVSALHRRIKAIVEVAAMCGVNIICFQEA
WTMPFAFCTREKLPWTEFAESAEDGPTTRFCQKLAKNHDMVVVSPILERDSEHGDVLWNT
AVVISNSGAVLGKTRKNHIPRVGDFNESTYYMEGNLGHPVFQTQFGRIAVNICYGRHHPL
NWLMYSINGAEIIFNPSATIGALSESLWPIEARNAAIANHCFTCAINRVGTEHFPNEFTS
GDGKKAHQDFGYFYGSSYVAAPDSSRTPGLSRSRDGLLVAKLDLNLCQQVNDVWNFKMTG
RYEMYARELAEAVKSNYSPTIVKE
Enzyme 1 Number of Residues 384
Enzyme 1 Molecular Weight 43166
Enzyme 1 Theoretical pI 6.51
Enzyme 1 GO Classification
Function
  • catalytic activity
  • hydrolase activity
  • hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds
Process
  • metabolism
  • nitrogen compound metabolism
  • physiological process
Component
Enzyme 1 General Function Not Available
Enzyme 1 Specific Function Not Available
Enzyme 1 Pathways Not Available
Enzyme 1 Reactions Not Available
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • None
Enzyme 1 Transmembrane Regions
  • None
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 126153365 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID A3KMF8 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name A3KMF8_HUMAN Link Image
Enzyme 1 PDB ID Not Available
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence Not Available
Enzyme 1 GenBank Gene ID BC131703 Link Image
Enzyme 1 GeneCard ID A3KMF8 Link Image
Enzyme 1 GenAtlas ID Not Available
Enzyme 1 HGNC ID Not Available
Enzyme 1 Chromosome Location Not Available
Enzyme 1 Locus Not Available
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References
  1. Strausberg RL, Feingold EA, Grouse LH, Derge JG, Klausner RD, Collins FS, Wagner L, Shenmen CM, Schuler GD, Altschul SF, Zeeberg B, Buetow KH, Schaefer CF, Bhat NK, Hopkins RF, Jordan H, Moore T, Max SI, Wang J, Hsieh F, Diatchenko L, Marusina K, Farmer AA, Rubin GM, Hong L, Stapleton M, Soares MB, Bonaldo MF, Casavant TL, Scheetz TE, Brownstein MJ, Usdin TB, Toshiyuki S, Carninci P, Prange C, Raha SS, Loquellano NA, Peters GJ, Abramson RD, Mullahy SJ, Bosak SA, McEwan PJ, McKernan KJ, Malek JA, Gunaratne PH, Richards S, Worley KC, Hale S, Garcia AM, Gay LJ, Hulyk SW, Villalon DK, Muzny DM, Sodergren EJ, Lu X, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madan A, Young AC, Shevchenko Y, Bouffard GG, Blakesley RW, Touchman JW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Krzywinski MI, Skalska U, Smailus DE, Schnerch A, Schein JE, Jones SJ, Marra MA: Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. Proc Natl Acad Sci U S A. 2002 Dec 24;99(26):16899-903. Epub 2002 Dec 11. [PubMed Link Image]
Enzyme 1 Metabolite References Not Available