We are currently updating the database - data may be missing for the next 10 minutes. We apologize for any inconvenience.

Human Metabolome Database Version 2.5

 

Showing metabocard for Ureidoisobutyric acid (HMDB02031)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2006-05-22 15:17:33
Update Date 2009-09-09 10:05:55
Accession Number HMDB02031
Secondary Accession Numbers Not Available
Common Name Ureidoisobutyric acid
Description Beta-ureidoisobutyrate (betaUIB)is increased in the urine of patients with beta-ureidopropionase deficiency [EC 3.5.1.6].(PMID: 12271438) Ureidoisobutyric acid can be used to predict patient's individual phenotypes of enzyme deficiencies in pyrimidine metabolism when associated with a risk for severe toxicity against the antineoplastic agent 5-fluorouracil. (PMID: 12798197)
Synonyms
  1. 3-Ureidoisobutyrate
  2. 3-carbamoylamino-2-methylpropanoate
  3. 3-carbamoylamino-2-methylpropanoic acid
  4. 3-ureidoisobutyric acid
  5. DL-beta-Ureidoisobutyrate
  6. DL-beta-Ureidoisobutyric acid
  7. beta-Uba
  8. beta-Ureidoisobutyrate
  9. beta-Ureidoisobutyric acid
  10. N-carbamyl-amino isobutyrate
  11. N-Carbamyl-b-aminoisobutyric acid
  12. N-Carbamyl-beta-aminoisobutyric acid
Chemical IUPAC Name 3-(carbamoylamino)-2-methyl-propanoic acid
Chemical Formula C5H10N2O3
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
  • carboxylic acid
  • urea
Biofunction
  • Protein synthesis, amino acid biosynthesis
Application
—
Source
  • Endogenous
Average Molecular Weight 146.145
Monoisotopic Molecular Weight 146.069138
Isomeric SMILES CC(CNC(N)=O)C(O)=O
Canonical SMILES CC(CNC(N)=O)C(O)=O
KEGG Compound ID C05100 Link Image
BioCyc ID 3-UREIDO-ISOBUTYRATE Link Image
BiGG ID 45118 Link Image
Wikipedia Link Not Available
NuGOwiki Link HMDB02031 Link Image
Metagene Link HMDB02031 Link Image
METLIN ID Not Available
PubChem Compound 160663 Link Image
PubChem Substance 738170 Link Image
ChEBI ID 1670 Link Image
CAS Registry Number 2905-86-4
InChI Identifier InChI=1/C5H10N2O3/c1-3(4(8)9)2-7-5(6)10/h3H,2H2,1H3,(H,8,9)(H3,6,7,10)
Synthesis Reference Evans, W. R.; Tsai, Chi Shiun; Axelrod, Bernard. Origin of b-aminoisobutyric acid in iris. Nature (London, United Kingdom) (1961), 190 809.
Melting Point (Experimental) Not Available
Experimental Water Solubility Not Available Source: PhysProp
Predicted Water Solubility 46.100002 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge -1
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity -0.86 [Predicted by ALOGPS]; -1.3 [Predicted by PubChem via XLOGP] Calculated using ALOGPS
Material Safety Data Sheet (MSDS) Not Available
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID Not Available
Experimental 1H NMR Spectrum Not Available
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Not Available
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
Show Peaklist
Mass Spectrum Not Available
Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • Cytoplasm (Predicted from LogP)
Biofluid Location
  • Cerebrospinal Fluid
  • Urine
Tissue Location Not Available
Concentrations (Normal)
Biofluid CSF
Value 0.01 +/- 0.05 uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • 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]
Biofluid Urine
Value 0.113 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Hofmann U, Schwab M, Seefried S, Marx C, Zanger UM, Eichelbaum M, Murdter TE: Sensitive method for the quantification of urinary pyrimidine metabolites in healthy adults by gas chromatography-tandem mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 5;791(1-2):371-80. [PubMed Link Image]
Concentrations (Abnormal)
Biofluid CSF
Value 1.4 - 5.9 uM
Age Adult:>18 yrs old
Sex Both
Condition Beta-ureidopropionase deficiency
Comments Not Available
References
  • 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]
Associated Disorders
Condition References
Beta-ureidopropionase deficiency
  • 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]
OMIM ID
  • 613161 Link Image (Beta-ureidopropionase deficiency)
Pathways
Name SMPDB Link KEGG Link
Pyrimidine Metabolism SMP00046 Link Image map00240 Link Image
General References
  1. Hofmann U, Schwab M, Seefried S, Marx C, Zanger UM, Eichelbaum M, Murdter TE: Sensitive method for the quantification of urinary pyrimidine metabolites in healthy adults by gas chromatography-tandem mass spectrometry. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 5;791(1-2):371-80. [PubMed Link Image]
  2. Ohse M, Matsuo M, Ishida A, Kuhara T: Screening and diagnosis of beta-ureidopropionase deficiency by gas chromatographic/mass spectrometric analysis of urine. J Mass Spectrom. 2002 Sep;37(9):954-62. [PubMed Link Image]
Metabolic Enzymes
  1. Dihydropyrimidinase
  2. UPB1 protein
Enzyme 1 [top]
Enzyme 1 ID 5814
Enzyme 1 Name Dihydropyrimidinase
Enzyme 1 Synonyms
  1. DHPase
  2. Hydantoinase
  3. DHP
Enzyme 1 Gene Name DPYS
Enzyme 1 Protein Sequence >Dihydropyrimidinase 
MAAPSRLLIRGGRVVNDDFSEVADVLVEDGVVRALGHDLLPPGGAPAGLRVLDAAGKLVL
PGGIDTHTHMQFPFMGSRSIDDFHQGTKAALSGGTTMIIDFAIPQKGGSLIEAFETWRSW
ADPKVCCDYSLHVAVTWWSDQVKEEMKILVQDKGVNSFKMFMAYKDLYMVTDLELYEAFS
RCKEIGAIAQVHAENGDLIAEGAKKMLALGITGPEGHELCRPEAVEAEATLRAITIASAV
NCPLYIVHVMSKSAAKVIADARRDGKVVYGEPIAASLGTDGTHYWNKEWHHAAHHVMGPP
LRPDPSTPDFLMNLLANDDLTTTGTDNCTFNTCQKALGKDDFTKIPNGVNGVEDRMSVIW
EKGVHSGKMDENRFVAVTSTNAAKIFNLYPRKGRIAVGSDADIVIWDPKGTRTISAKTHH
QAVNFNIFEGMVCHGVPLVTISRGKVVYEAGVFSVTAGDGKFIPRKPFAEYIYKRIKQRD
RTCTPTPVERAPYKGEVATLKSRVTKEDATAGTRKQAHP
Enzyme 1 Number of Residues 519
Enzyme 1 Molecular Weight 56630
Enzyme 1 Theoretical pI 7.28
Enzyme 1 GO Classification
Function
  • catalytic activity
  • hydrolase activity
Process
—
Component
—
Enzyme 1 General Function Nucleotide transport and metabolism
Enzyme 1 Specific Function 5,6-dihydrouracil + H(2)O = 3- ureidopropanoate
Enzyme 1 Pathways
Enzyme 1 Reactions
  • 5,6-dihydrouracil + H2O = 3-ureidopropanoate
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • 1-2
Enzyme 1 Transmembrane Regions Not Available
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 1330236 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID Q14117 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name DPYS_HUMAN Link Image
Enzyme 1 PDB ID Not Available
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence >1560 bp 
ATGGCGGCGCCCTCGCGGCTCCTGATCCGCGGGGGTCGCGTGGTCAACGATGACTTCTCG
GAGGTGGCCGACGTGCTGGTGGAGGACGGCGTGGTGCGGGCACTCGGGCACGACCTGCTG
CCTCCCGGGGGCGCTCCTGCGGGGCTGCGGGTCCTCGACGCCGCCGGCAAGCTCGTCCTG
CCCGGAGGCATCGACACACACACGCACATGCAGTTCCCCTTCATGGGCTCGCGGTCCATC
GACGACTTCCACCAGGGCACCAAGGCTGCTCTCTCAGGAGGCACCACCATGATTATTGAT
TTCGCCATTCCTCAGAAAGGTGGCTCCCTCATTGAGGCCTTCGAGACCTGGCGAAGCTGG
GCTGATCCCAAAGTTTGCTGCGACTACAGCCTTCATGTGGCAGTGACGTGGTGGAGTGAC
CAGGTTAAAGAAGAAATGAAAATCCTTGTGCAAGATAAAGGTGTTAACTCTTTCAAGATG
TTTATGGCCTATAAAGATCTGTACATGGTGACAGACCTGGAGCTGTACGAAGCCTTCTCT
CGGTGCAAGGAAATTGGAGCAATTGCCCAGGTCCATGCGGAAAATGGAGACTTAATTGCA
GAGGGAGCAAAGAAGATGTTGGCTCTGGGGATAACAGGCCCTGAGGGCCACGAGCTGTGC
CGCCCAGAGGCAGTGGAGGCAGAGGCCACGCTGAGAGCCATCACCATAGCCAGCGCTGTG
AACTGTCCTCTCTACATTGTGCATGTGATGAGCAAGTCTGCAGCTAAGGTGATAGCGGAT
GCAAGGAGAGATGGGAAGGTGGTCTATGGTGAACCCATAGCAGCCAGTCTTGGCACAGAT
GGCACTCACTACTGGAATAAAGAATGGCACCATGCAGCCCACCATGTCATGGGTCCACCT
TTGCGACCAGACCCCTCAACACCCGACTTCCTCATGAATCTGTTGGCTAATGATGATCTA
ACCACAACAGGGACTGATAACTGCACTTTCAACACCTGCCAGAAAGCTCTTGGGAAGGAT
GATTTTACCAAGATCCCCAATGGGGTGAATGGTGTTGAAGATCGGATGTCCGTAATATGG
GAAAAAGGCGTGCATAGTGGTAAAATGGATGAAAACAGATTTGTGGCAGTTACCAGCACA
AATGCAGCCAAAATTTTTAATCTCTATCCAAGAAAAGGAAGAATAGCTGTAGGATCAGAT
GCTGACATTGTTATTTGGGACCCAAAAGGCACAAGGACTATCTCAGCAAAAACTCATCAT
CAGGCTGTTAACTTCAACATTTTCGAGGGCATGGTTTGCCACGGGGTGCCCCTTGTGACT
ATTTCAAGAGGCAAAGTGGTATATGAAGCCGGAGTGTTCAGTGTCACGGCAGGAGATGGG
AAGTTTATTCCTCGAAAACCATTTGCTGAATATATTTACAAACGAATAAAGCAGCGAGAC
CGGACTTGCACACCTACCCCTGTGGAGCGTGCACCCTATAAGGGAGAAGTCGCCACACTG
AAATCCAGAGTGACAAAAGAAGATGCCACAGCAGGGACCAGGAAACAGGCCCACCCCTGA
Enzyme 1 GenBank Gene ID D78011 Link Image
Enzyme 1 GeneCard ID DPYS Link Image
Enzyme 1 GenAtlas ID DPYS Link Image
Enzyme 1 HGNC ID HGNC:3013 Link Image
Enzyme 1 Chromosome Location 8
Enzyme 1 Locus 8q22
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References
  1. Hamajima N, Matsuda K, Sakata S, Tamaki N, Sasaki M, Nonaka M: A novel gene family defined by human dihydropyrimidinase and three related proteins with differential tissue distribution. Gene. 1996 Nov 21;180(1-2):157-63. [PubMed Link Image]
  2. Hamajima N, Kouwaki M, Vreken P, Matsuda K, Sumi S, Imaeda M, Ohba S, Kidouchi K, Nonaka M, Sasaki M, Tamaki N, Endo Y, De Abreu R, Rotteveel J, van Kuilenburg A, van Gennip A, Togari H, Wada Y: Dihydropyrimidinase deficiency: structural organization, chromosomal localization, and mutation analysis of the human dihydropyrimidinase gene. Am J Hum Genet. 1998 Sep;63(3):717-26. [PubMed Link Image]
Enzyme 1 Metabolite References Not Available
Enzyme 2 [top]
Enzyme 2 ID 12999
Enzyme 2 Name UPB1 protein
Enzyme 2 Synonyms
  1. Ureidopropionase, beta, isoform CRA_b
Enzyme 2 Gene Name UPB1
Enzyme 2 Protein Sequence >UPB1 protein 
MAGAEWKSLEECLEKHLPLPDLQEVKRVLYGKELRKLDLPREAFEAASREDFELQGYAFE
AAEEQLRRPRIVHVGLVQNRIPLPANAPVAEQVSALHRRIKAIVEVAAMCGVNIICFQEA
WTMPFAFCTREKLPWTEFAESAEDGPTTRFCQKLAKNHDMVVVSPILERDSEHGDVLWNT
AVVISNSGAVLGKTRKNHIPRVGDFNESTYYMEGNLGHPVFQTQFGRIAVNICYGRHHPL
NWLMYSINGAEIIFNPSATIGALSESLWPIEARNAAIANHCFTCAINRVGTEHFPNEFTS
GDGKKAHQDFGYFYGSSYVAAPDSSRTPGLSRSRDGLLVAKLDLNLCQQVNDVWNFKMTG
RYEMYARELAEAVKSNYSPTIVKE
Enzyme 2 Number of Residues 384
Enzyme 2 Molecular Weight 43166
Enzyme 2 Theoretical pI 6.51
Enzyme 2 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 2 General Function Not Available
Enzyme 2 Specific Function Not Available
Enzyme 2 Pathways Not Available
Enzyme 2 Reactions Not Available
Enzyme 2 Pfam Domain Function
Enzyme 2 Signals
  • None
Enzyme 2 Transmembrane Regions
  • None
Enzyme 2 Essentiality Not Available
Enzyme 2 GenBank ID Protein 126153365 Link Image
Enzyme 2 UniProtKB/Swiss-Prot ID A3KMF8 Link Image
Enzyme 2 UniProtKB/Swiss-Prot Entry Name A3KMF8_HUMAN Link Image
Enzyme 2 PDB ID Not Available
Enzyme 2 Cellular Location Not Available
Enzyme 2 Gene Sequence Not Available
Enzyme 2 GenBank Gene ID BC131703 Link Image
Enzyme 2 GeneCard ID A3KMF8 Link Image
Enzyme 2 GenAtlas ID Not Available
Enzyme 2 HGNC ID Not Available
Enzyme 2 Chromosome Location Not Available
Enzyme 2 Locus Not Available
Enzyme 2 SNPs SNPJam Report Link Image
Enzyme 2 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 2 Metabolite References Not Available