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Identification
HMDB Protein ID HMDBP00914
Secondary Accession Numbers
  • 6202
Name Urocanate hydratase
Synonyms
  1. Imidazolonepropionate hydrolase
  2. Urocanase
Gene Name UROC1
Protein Type Unknown
Biological Properties
General Function Involved in urocanate hydratase activity
Specific Function Not Available
Pathways
  • Histidine Metabolism
  • Histidine metabolism
  • Histidinemia
  • L-histidine degradation into L-glutamate
Reactions
4-Imidazolone-5-propionic acid → Urocanic acid + Water details
GO Classification
Biological Process
histidine catabolic process to glutamate and formamide
histidine catabolic process to glutamate and formate
histidine catabolic process
Cellular Component
cytosol
Function
catalytic activity
lyase activity
urocanate hydratase activity
carbon-oxygen lyase activity
hydro-lyase activity
Molecular Function
urocanate hydratase activity
Process
metabolic process
cellular metabolic process
cellular amino acid and derivative metabolic process
cellular amino acid metabolic process
histidine family amino acid metabolic process
histidine metabolic process
histidine catabolic process
Cellular Location Not Available
Gene Properties
Chromosome Location 3
Locus 3q21.3
SNPs UROC1
Gene Sequence
>2031 bp
ATGTCTAGCCTCCAGGCGCTGTGCTCTGGCCTGCCCCTGCGGCCCCTCCCAGAGAACCGG
GGACGCCAGGCTGGGGTGCCCCATGCCCCTGTCAGGACCCCCAGCCTCAGCCCTGTGGAG
AAACAGCTGGCGCTGAGGAACGCCCTGCGCTACTTCCCCCCGGATGTCCAGGAGCTGCTG
GCCCCAGAGTTTGCCCAGGAGCTGCAACTGTACGGACACATCTACATGTACCGGTTTTGC
CCCGACATTGAAATGAGGGCCTACCCGATTGAGCAGTACCCCTGCCAGACGAAAGTGGCT
GCCGCCATCATGCACATGATTATGAACAACCTGGATCCTGCCGTGGCCCAGTTTCCCCAG
GAGCTGGTGACCTATGGAGGAAATGGGCAGGTGTTCAGCAACTGGGCTCAGTTCTGGCTG
ACCATGTTCTACTTGTCGAAGATGACAGAGGAGCAGACTTTGGTCATGTACAGTGGGCAC
CCACTTGGCCTCTTTCCCAGCAGCCGCAGTGCCCCACGGCTCGTCATCACCAATGGGATG
GTCATTCCCAACTACTCCTCCCGGACGGAGTATGAGAAGCTCTTTGCCTTGGGGGTTACA
ATGTACGGCCAGATGACAGCAGGTAGCTACTGCTACATCGGTCCCCAGGGAATCGTTCAT
GGCACTGTGCTCACCGTGTTGAATGCTGCACGTCGGTACCTGGGCATCGAGGACTTGGCT
GGGAAGGTCTTTGTCACCTCTGGGCTCGGCGGAATGAGTGGGGCTCAGGCCAAGGCCGCA
GTCATCGTGGGGTGCATCGGTGTGATAGCAGAGGTGGATAAAGCAGCCCTTGAGAAACGC
CACAGGCAGGGCTGGCTGATGGAAGTGACTGACAGCTTGGACCGCTGCATCCAGAGGCTC
AGGGAAGCAAGGAAAAAAAAGGAGGTGCTCAGCCTTGGTTACCATGGCAACGTGGTGGCT
CTTTGGGAGCGCCTGGTCCACGAATTGGACACGACGGGGGAGTGCTTGGTGGACCTGGGG
TCAGATCAGACATCCTGCCACAACCCGTTCAATGGCGGCTACTACCCTGTGCAGCTCAGC
TTCACGGAGGCCCAGAGCCTCATGGCCTCCAACCCTGCTGTGTTCAAGGACCTGGTCCAG
GAAAGCCTGAGGAGGCAAGTCTCAGCCATCAACAGGTTGGCCGAGGAGAAGTTCTTCTTC
TGGGACTACGGCAATGCCTTCCTCTTGGAGGCCCAGAGAGCAGGAGCGGATGTGGAGAAG
AAAGGTGCTGGCAGGACAGAGTTCCGCTACCCTTCCTATGTGCAGCACATCATGGGGGAC
ATATTCTCCCAGGGATTTGGGCCTTTCCGCTGGGTGTGCACATCGGGGGACCCCCAGGAC
CTGGCGGTCACAGACGAACTGGCCACATCTGTGCTGGAGGAAGCCATTGCTGATGGAGTG
AAGGTGTCTGTGAAGCTGCAGTACATGGACAACATCCGCTGGATCCGGGAGGCCGCCAGG
CACCGGCTGGTGGTGGGCTCCCAGGCAAGGATCTTGTACTCAGACCAGAAGGGCCGCGTG
GCCATCGCTGTGGCCATTAACCAGGCCATCGCCTGCAGGAGGATCAAGGCGCCGGTGGTC
CTGAGCCGAGATCACCATGACGTGAGCGGCACCGACAGCCCCTTTAGGGAGACCTCCAAC
ATTTACGACGGCTCTGCCTTCTGTGCAGACATGGCTGTGCAGAACTTCGTGGGAGATGCC
TGTCGCGGAGCCACCTGGGTCGCCCTTCACAACGGAGGGGGCGTGGGCTGGGGTGAGGTG
ATCAACGGGGGATTCGGCCTCGTGCTGGACGGTACCCCGGAGGCCGAGGGGAGAGCCAGG
CTGATGCTCAGCTGGGATGTCTCCAATGGTGTGGCCCGGCGCTGCTGGTCAGGGAACCAG
AAGGCCTATGAGATCATCTGCCAGACCATGCAGGAGAACAGCACCTTGGTGGTGACACTG
CCTCACAAGGTGGAGGACGAGCGGGTGCTCCAGCAGGCCCTGCAGCTCTGA
Protein Properties
Number of Residues 676
Molecular Weight 80911.275
Theoretical pI 7.011
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Urocanate hydratase
MSSLQALCSGLPLRPLPENRGRQAGVPHAPVRTPSLSPVEKQLALRNALRYFPPDVQELL
APEFAQELQLYGHIYMYRFCPDIEMRAYPIEQYPCQTKVAAAIMHMIMNNLDPAVAQFPQ
ELVTYGGNGQVFSNWAQFWLTMFYLSKMTEEQTLVMYSGHPLGLFPSSRSAPRLVITNGM
VIPNYSSRTEYEKLFALGVTMYGQMTAGSYCYIGPQGIVHGTVLTVLNAARRYLGIEDLA
GKVFVTSGLGGMSGAQAKAAVIVGCIGVIAEVDKAALEKRHRQGWLMEVTDSLDRCIQRL
REARKKKEVLSLGYHGNVVALWERLVHELDTTGECLVDLGSDQTSCHNPFNGGYYPVQLS
FTEAQSLMASNPAVFKDLVQESLRRQVSAINRLAEEKFFFWDYGNAFLLEAQRAGADVEK
KGAGRTEFRYPSYVQHIMGDIFSQGFGPFRWVCTSGDPQDLAVTDELATSVLEEAIADGV
KVSVKLQYMDNIRWIREAARHRLVVGSQARILYSDQKGRVAIAVAINQAIACRRIKAPVV
LSRDHHDVSGTDSPFRETSNIYDGSAFCADMAVQNFVGDACRGATWVALHNGGGVGWGEV
INGGFGLVLDGTPEAEGRARLMLSWDVSNGVARRCWSGNQKAYEIICQTMQENSTLVVTL
PHKVEDERVLQQALQL
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID Q96N76
UniProtKB/Swiss-Prot Entry Name HUTU_HUMAN
PDB IDs Not Available
GenBank Gene ID AK055862
GeneCard ID UROC1
GenAtlas ID UROC1
HGNC ID HGNC:26444
References
General References
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  2. 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:15489334 ]
  3. Espinos C, Pineda M, Martinez-Rubio D, Lupo V, Ormazabal A, Vilaseca MA, Spaapen LJ, Palau F, Artuch R: Mutations in the urocanase gene UROC1 are associated with urocanic aciduria. J Med Genet. 2009 Jun;46(6):407-11. doi: 10.1136/jmg.2008.060632. Epub 2009 Mar 19. [PubMed:19304569 ]