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

• Cytoplasm (Predicted from LogP)

• Cerebrospinal Fluid
• Urine

• 613161 (Beta-ureidopropionase deficiency)

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 ]
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 ]

1. Dihydropyrimidinase
2. Beta-ureidopropionase
3. UPB1 protein

1. DHP
2. DHPase
3. Dihydropyrimidine amidohydrolase
4. Hydantoinase

MAAPSRLLIRGGRVVNDDFSEVADVLVEDGVVRALGHDLLPPGGAPAGLRVLDAAGKLVL
PGGIDTHTHMQFPFMGSRSIDDFHQGTKAALSGGTTMIIDFAIPQKGGSLIEAFETWRSW
ADPKVCCDYSLHVAVTWWSDQVKEEMKILVQDKGVNSFKMFMAYKDLYMVTDLELYEAFS
RCKEIGAIAQVHAENGDLIAEGAKKMLALGITGPEGHELCRPEAVEAEATLRAITIASAV
NCPLYIVHVMSKSAAKVIADARRDGKVVYGEPIAASLGTDGTHYWNKEWHHAAHHVMGPP
LRPDPSTPDFLMNLLANDDLTTTGTDNCTFNTCQKALGKDDFTKIPNGVNGVEDRMSVIW
EKGVHSGKMDENRFVAVTSTNAAKIFNLYPRKGRIAVGSDADIVIWDPKGTRTISAKTHH
QAVNFNIFEGMVCHGVPLVTISRGKVVYEAGVFSVTAGDGKFIPRKPFAEYIYKRIKQRD
RTCTPTPVERAPYKGEVATLKSRVTKEDATAGTRKQAHP

• Beta Alanine Metabolism (map00410 )
• Pantothenate and CoA Biosynthesis (map00770 )
• Pyrimidine Metabolism (map00240 )

• 5,6-dihydrouracil + H2O = 3-ureidopropanoate [RN:R02269]

• Amidohydro_1 (PF01979 )

• None

• None

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

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 ]
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 ]
3. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed ]

1. BUP-1
2. Beta-alanine synthase
3. N-carbamoyl-beta-alanine amidohydrolase

MAGAEWKSLEECLEKHLPLPDLQEVKRVLYGKELRKLDLPREAFEAASREDFELQGYAFE
AAEEQLRRPRIVHVGLVQNRIPLPANAPVAEQVSALHRRIKAIVEVAAMCGVNIICFQEA
WTMPFAFCTREKLPWTEFAESAEDGPTTRFCQKLAKNHDMVVVSPILERDSEHGDVLWNT
AVVISNSGAVLGKTRKNHIPRVGDFNESTYYMEGNLGHPVFQTQFGRIAVNICYGRHHPL
NWLMYSINGAEIIFNPSATIGALSESLWPIEARNAAIANHCFTCAINRVGTEHFPNEFTS
GDGKKAHQDFGYFYGSSYVAAPDSSRTPGLSRSRDGLLVAKLDLNLCQQVNDVWNFKMTG
RYEMYARELAEAVKSNYSPTIVKE

• Beta Alanine Metabolism (map00410 )
• Pantothenate and CoA Biosynthesis (map00770 )
• Pyrimidine Metabolism (map00240 )

• N-carbamoyl-beta-alanine + H2O = beta-alanine + CO2 + NH3 [RN:R00905]

• CN_hydrolase (PF00795 )

• None

• None

ATGGCGGGCGCTGAGTGGAAGTCGCTGGAGGAATGCTTGGAGAAGCACCTGCCGCTCCCC
GACTTGCAGGAAGTGAAGCGCGTTCTCTATGGCAAGGAACTCAGGAAGCTTGATCTGCCC
AGGGAAGCTTTCGAAGCTGCCTCCAGAGAAGACTTTGAACTGCAGGGATATGCCTTTGAA
GCAGCGGAGGAGCAGCTGAGACGACCCCGCATTGTGCACGTGGGGCTGGTTCAGAACAGA
ATCCCCCTCCCCGCAAATGCCCCTGTGGCAGAACAGGTCTCTGCCCTTCATAGACGCATA
AAGGCTATCGTAGAGGTGGCTGCAATGTGTGGAGTCAACATCATCTGTTTCCAGGAAGCA
TGGACTATGCCCTTTGCCTTCTGTACGAGAGAGAAGCTTCCTTGGACAGAATTTGCTGAG
TCAGCAGAGGATGGGCCCACCACCAGATTCTGTCAGAAGCTGGCGAAGAACCATGACATG
GTGGTGGTGTCTCCCATCCTGGAACGAGACAGCGAGCATGGGGATGTTTTGTGGAATACA
GCCGTGGTGATCTCCAATTCCGGAGCAGTCCTGGGAAAGACCAGGAAAAACCACATCCCC
AGAGTGGGTGATTTCAACGAGTCAACTTACTACATGGAGGGAAACCTGGGCCACCCCGTG
TTCCAGACGCAGTTCGGAAGGATCGCGGTGAACATTTGCTACGGGCGGCACCACCCCCTC
AACTGGCTTATGTACAGCATCAACGGGGCTGAGATCATCTTCAACCCCTCGGCCACGATA
GGAGCACTCAGCGAGTCCCTGTGGCCCATCGAGGCCAGAAACGCAGCCATTGCCAATCAC
TGCTTCACCTGCGCCATCAATCGAGTGGGCACCGAGCACTTCCCGAACGAGTTTACCTCG
GGAGATGGAAAGAAAGCTCACCAGGACTTTGGCTACTTTTATGGCTCGAGCTATGTGGCA
GCCCCTGACAGCAGCCGGACTCCTGGGCTGTCCCGTAGCCGGGATGGACTGCTAGTTGCT
AAGCTCGACCTAAACCTCTGCCAGCAGGTGAATGATGTCTGGAACTTCAAGATGACGGGC
AGGTATGAGATGTACGCACGGGAGCTCGCCGAAGCTGTCAAGTCCAACTACAGCCCCACC
ATCGTGAAAGAGTAG

1. Vreken P, van Kuilenburg AB, Hamajima N, Meinsma R, van Lenthe H, Gohlich-Ratmann G, Assmann BE, Wevers RA, van Gennip AH: cDNA cloning, genomic structure and chromosomal localization of the human BUP-1 gene encoding beta-ureidopropionase. Biochim Biophys Acta. 1999 Oct 28;1447(2-3):251-7. [PubMed ]
2. Sakamoto T, Sakata SF, Matsuda K, Horikawa Y, Tamaki N: Expression and properties of human liver beta-ureidopropionase. J Nutr Sci Vitaminol (Tokyo). 2001 Apr;47(2):132-8. [PubMed ]
3. Collins JE, Wright CL, Edwards CA, Davis MP, Grinham JA, Cole CG, Goward ME, Aguado B, Mallya M, Mokrab Y, Huckle EJ, Beare DM, Dunham I: A genome annotation-driven approach to cloning the human ORFeome. Genome Biol. 2004;5(10):R84. Epub 2004 Sep 30. [PubMed ]
4. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed ]
5. 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 ]

1. Ureidopropionase, beta, isoform CRA_b

MAGAEWKSLEECLEKHLPLPDLQEVKRVLYGKELRKLDLPREAFEAASREDFELQGYAFE
AAEEQLRRPRIVHVGLVQNRIPLPANAPVAEQVSALHRRIKAIVEVAAMCGVNIICFQEA
WTMPFAFCTREKLPWTEFAESAEDGPTTRFCQKLAKNHDMVVVSPILERDSEHGDVLWNT
AVVISNSGAVLGKTRKNHIPRVGDFNESTYYMEGNLGHPVFQTQFGRIAVNICYGRHHPL
NWLMYSINGAEIIFNPSATIGALSESLWPIEARNAAIANHCFTCAINRVGTEHFPNEFTS
GDGKKAHQDFGYFYGSSYVAAPDSSRTPGLSRSRDGLLVAKLDLNLCQQVNDVWNFKMTG
RYEMYARELAEAVKSNYSPTIVKE

• CN_hydrolase (PF00795 )

• None

• None

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 ]