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{{Infobox_gene}}
{{PBB_Controls
'''Beta-ureidopropionase''' is an [[enzyme]] that in humans is encoded by the ''UPB1'' [[gene]].<ref name="pmid10542323">{{cite journal |vauthors=Vreken P, van Kuilenburg AB, Hamajima N, Meinsma R, van Lenthe H, Gohlich-Ratmann G, Assmann BE, Wevers RA, van Gennip AH | title = cDNA cloning, genomic structure and chromosomal localization of the human BUP-1 gene encoding beta-ureidopropionase | journal = Biochim Biophys Acta | volume = 1447 | issue = 2–3 | pages = 251–7 |date=Dec 1999 | pmid = 10542323 | pmc =  | doi = 10.1016/s0167-4781(99)00182-7}}</ref><ref name="entrez">{{cite web | title = Entrez Gene: UPB1 ureidopropionase, beta| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=51733| accessdate = }}</ref>
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<!-- The GNF_Protein_box is automatically maintained by Protein Box Bot. See Template:PBB_Controls to Stop updates. -->
{{GNF_Protein_box
| image =
| image_source =
| PDB =
| Name = Ureidopropionase, beta
| HGNCid = 16297
| Symbol = UPB1
| AltSymbols =; BUP1
| OMIM = 606673
| ECnumber =
| Homologene = 9471
| MGIid = 2143535
  | GeneAtlas_image1 = PBB_GE_UPB1_220507_s_at_tn.png
| Function = {{GNF_GO|id=GO:0003837 |text = beta-ureidopropionase activity}}{{GNF_GO|id=GO:0008270 |text = zinc ion binding}}{{GNF_GO|id=GO:0016810 |text = hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds}}{{GNF_GO|id=GO:0046872 |text = metal ion binding}}
| Component =
| Process = {{GNF_GO|id=GO:0006807 |text = nitrogen compound metabolic process}}
| Orthologs = {{GNF_Ortholog_box
    | Hs_EntrezGene = 51733
    | Hs_Ensembl = ENSG00000100024
    | Hs_RefseqProtein = NP_057411
    | Hs_RefseqmRNA = NM_016327
    | Hs_GenLoc_db = 
    | Hs_GenLoc_chr = 22
    | Hs_GenLoc_start = 23221251
    | Hs_GenLoc_end = 23252552
    | Hs_Uniprot = Q9UBR1
    | Mm_EntrezGene = 103149
    | Mm_Ensembl = ENSMUSG00000033427
    | Mm_RefseqmRNA = NM_133995
    | Mm_RefseqProtein = NP_598756
    | Mm_GenLoc_db = 
    | Mm_GenLoc_chr = 10
    | Mm_GenLoc_start = 74850749
    | Mm_GenLoc_end = 74883913
    | Mm_Uniprot = Q3UEK4
  }}
}}
 
 
==Overview==
'''Ureidopropionase, beta''', also known as '''UPB1''', is a human [[gene]].<ref name="entrez">{{cite web | title = Entrez Gene: UPB1 ureidopropionase, beta| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=51733| accessdate = }}</ref>


<!-- The PBB_Summary template is automatically maintained by Protein Box Bot.  See Template:PBB_Controls to Stop updates. -->
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{{PBB_Summary
{{PBB_Summary
| section_title =  
| section_title =  
| summary_text = This gene encodes a protein that belongs to the CN hydrolase family. Beta-ureidopropionase catalyzes the last step in the pyrimidine degradation pathway. The pyrimidine bases uracil and thymine are degraded via the consecutive action of dihydropyrimidine dehydrogenase (DHPDH), dihydropyrimidinase (DHP) and beta-ureidopropionase (UP) to beta-alanine and beta-aminoisobutyric acid, respectively. UP deficiencies are associated with N-carbamyl-beta-amino aciduria and may lead to abnormalities in neurological activity.<ref name="entrez">{{cite web | title = Entrez Gene: UPB1 ureidopropionase, beta| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=51733| accessdate = }}</ref>
| summary_text = This gene encodes a protein that belongs to the CN hydrolase family. Beta-ureidopropionase catalyzes the last step in the pyrimidine degradation pathway. The pyrimidine bases [[uracil]] and [[thymine]] are degraded via the consecutive action of [[dihydropyrimidine dehydrogenase]] (DHPDH), [[dihydropyrimidinase]] (DHP) and [[beta-ureidopropionase]] (UP) to [[beta-alanine]] and [[beta-aminoisobutyric acid]], respectively. UP deficiencies are associated with N-carbamyl-beta-amino aciduria and may lead to abnormalities in neurological activity.<ref name="entrez" />
}}
}}
== Interactive pathway map ==
{{FluoropyrimidineActivity WP1601|highlight=UPB1}}


==References==
==References==
{{reflist}}
{{reflist}}
==Further reading==
==Further reading==
{{refbegin | 2}}
{{refbegin | 2}}
{{PBB_Further_reading  
{{PBB_Further_reading  
| citations =  
| citations =  
*{{cite journal  | author=Thomas HR, Ezzeldin HH, Guarcello V, ''et al.'' |title=Genetic regulation of beta-ureidopropionase and its possible implication in altered uracil catabolism. |journal=Pharmacogenet. Genomics |volume=18 |issue= 1 |pages= 25-35 |year= 2008 |pmid= 18216719 |doi= 10.1097/FPC.0b013e3282f2f134 }}
*{{cite journal  | author=Thomas HR |title=Genetic regulation of beta-ureidopropionase and its possible implication in altered uracil catabolism |journal=Pharmacogenet. Genomics |volume=18 |issue= 1 |pages= 25–35 |year= 2008 |pmid= 18216719 |doi= 10.1097/FPC.0b013e3282f2f134 |name-list-format=vanc| author2=Ezzeldin HH  | author3=Guarcello V  | display-authors=3  | last4=Mattison  | first4=Lori K.  | last5=Fridley  | first5=Brooke L.  | last6=Diasio  | first6=Robert B. }}
*{{cite journal  | author=Thomas HR, Ezzeldin HH, Guarcello V, ''et al.'' |title=Genetic regulation of dihydropyrimidinase and its possible implication in altered uracil catabolism. |journal=Pharmacogenet. Genomics |volume=17 |issue= 11 |pages= 973-87 |year= 2008 |pmid= 18075467 |doi= 10.1097/FPC.0b013e3282f01788 }}
*{{cite journal  | author=Thomas HR |title=Genetic regulation of dihydropyrimidinase and its possible implication in altered uracil catabolism |journal=Pharmacogenet. Genomics |volume=17 |issue= 11 |pages= 973–87 |year= 2008 |pmid= 18075467 |doi= 10.1097/FPC.0b013e3282f01788 |name-list-format=vanc| author2=Ezzeldin HH  | author3=Guarcello V  | display-authors=3  | last4=Mattison  | first4=Lori K.  | last5=Fridley  | first5=Brooke L.  | last6=Diasio  | first6=Robert B. }}
*{{cite journal  | author=van Kuilenburg AB, Meinsma R, Assman B, ''et al.'' |title=Genetic analysis of the first 4 patients with beta-ureidopropionase deficiency. |journal=Nucleosides Nucleotides Nucleic Acids |volume=25 |issue= 9-11 |pages= 1093-8 |year= 2007 |pmid= 17065070 |doi= 10.1080/15257770600956870 }}
*{{cite journal  | author=van Kuilenburg AB |title=Genetic analysis of the first 4 patients with beta-ureidopropionase deficiency |journal=Nucleosides Nucleotides Nucleic Acids |volume=25 |issue= 9–11 |pages= 1093–8 |year= 2007 |pmid= 17065070 |doi= 10.1080/15257770600956870 |name-list-format=vanc| author2=Meinsma R  | author3=Assman B  | display-authors=3  | last4=Hoffman  | first4=G.  | last5=Voit  | first5=T.  | last6=Ribes  | first6=A.  | last7=Lorente  | first7=I.  | last8=Busch  | first8=R.  | last9=Mayatepek  | first9=E. }}
*{{cite journal  | author=Collins JE, Wright CL, Edwards CA, ''et al.'' |title=A genome annotation-driven approach to cloning the human ORFeome. |journal=Genome Biol. |volume=5 |issue= 10 |pages= R84 |year= 2005 |pmid= 15461802 |doi= 10.1186/gb-2004-5-10-r84 }}
*{{cite journal  | author=Collins JE |title=A genome annotation-driven approach to cloning the human ORFeome |journal=Genome Biol. |volume=5 |issue= 10 |pages= R84 |year= 2005 |pmid= 15461802 |doi= 10.1186/gb-2004-5-10-r84 | pmc=545604  |name-list-format=vanc| author2=Wright CL  | author3=Edwards CA  | display-authors=3  | last4=Davis  | first4=Matthew P  | last5=Grinham  | first5=James A  | last6=Cole  | first6=Charlotte G  | last7=Goward  | first7=Melanie E  | last8=Aguado  | first8=Begoña  | last9=Mallya  | first9=Meera }}
*{{cite journal  | author=van Kuilenburg AB, Meinsma R, Beke E, ''et al.'' |title=beta-Ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities. |journal=Hum. Mol. Genet. |volume=13 |issue= 22 |pages= 2793-801 |year= 2006 |pmid= 15385443 |doi= 10.1093/hmg/ddh303 }}
*{{cite journal  | author=van Kuilenburg AB |title=beta-Ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities |journal=Hum. Mol. Genet. |volume=13 |issue= 22 |pages= 2793–801 |year= 2006 |pmid= 15385443 |doi= 10.1093/hmg/ddh303 |name-list-format=vanc| author2=Meinsma R  | author3=Beke E  | display-authors=3  | last4=Assmann  | first4=B  | last5=Ribes  | first5=A  | last6=Lorente  | first6=I  | last7=Busch  | first7=R  | last8=Mayatepek  | first8=E  | last9=Abeling  | first9=NG }}
*{{cite journal  | author=Ota T, Suzuki Y, Nishikawa T, ''et al.'' |title=Complete sequencing and characterization of 21,243 full-length human cDNAs. |journal=Nat. Genet. |volume=36 |issue= 1 |pages= 40-5 |year= 2004 |pmid= 14702039 |doi= 10.1038/ng1285 }}
*{{cite journal  | author=Ota T |title=Complete sequencing and characterization of 21,243 full-length human cDNAs |journal=Nat. Genet. |volume=36 |issue= 1 |pages= 40–5 |year= 2004 |pmid= 14702039 |doi= 10.1038/ng1285 |name-list-format=vanc| author2=Suzuki Y  | author3=Nishikawa T  | display-authors=3  | last4=Otsuki  | first4=Tetsuji  | last5=Sugiyama  | first5=Tomoyasu  | last6=Irie  | first6=Ryotaro  | last7=Wakamatsu  | first7=Ai  | last8=Hayashi  | first8=Koji  | last9=Sato  | first9=Hiroyuki }}
*{{cite journal  | author=Strausberg RL, Feingold EA, Grouse LH, ''et al.'' |title=Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences. |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=99 |issue= 26 |pages= 16899-903 |year= 2003 |pmid= 12477932 |doi= 10.1073/pnas.242603899 }}
*{{cite journal  | author=Strausberg RL |title=Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=99 |issue= 26 |pages= 16899–903 |year= 2003 |pmid= 12477932 |doi= 10.1073/pnas.242603899 | pmc=139241  |name-list-format=vanc| author2=Feingold EA  | author3=Grouse LH  | display-authors=3  | last4=Derge  | first4=JG  | last5=Klausner  | first5=RD  | last6=Collins  | first6=FS  | last7=Wagner  | first7=L  | last8=Shenmen  | first8=CM  | last9=Schuler  | first9=GD }}
*{{cite journal  | author=Sakamoto T, Sakata SF, Matsuda K, ''et al.'' |title=Expression and properties of human liver beta-ureidopropionase. |journal=J. Nutr. Sci. Vitaminol. |volume=47 |issue= 2 |pages= 132-8 |year= 2002 |pmid= 11508704 |doi=  }}
*{{cite journal  | author=Sakamoto T |title=Expression and properties of human liver beta-ureidopropionase |journal=J. Nutr. Sci. Vitaminol. |volume=47 |issue= 2 |pages= 132–8 |year= 2002 |pmid= 11508704 |doi=  10.3177/jnsv.47.132|name-list-format=vanc| author2=Sakata SF  | author3=Matsuda K  | display-authors=3 | last4=Horikawa  | first4=Y  | last5=Tamaki  | first5=N }}
*{{cite journal  | author=Vreken P, van Kuilenburg AB, Hamajima N, ''et al.'' |title=cDNA cloning, genomic structure and chromosomal localization of the human BUP-1 gene encoding beta-ureidopropionase. |journal=Biochim. Biophys. Acta |volume=1447 |issue= 2-3 |pages= 251-7 |year= 1999 |pmid= 10542323 |doi=  }}
*{{cite journal  |vauthors=Naguib FN, el Kouni MH, Cha S |title=Enzymes of uracil catabolism in normal and neoplastic human tissues |journal=Cancer Res. |volume=45 |issue= 11 Pt 1 |pages= 5405–12 |year= 1985 |pmid= 3931905 |doi=  }}
*{{cite journal  | author=Naguib FN, el Kouni MH, Cha S |title=Enzymes of uracil catabolism in normal and neoplastic human tissues. |journal=Cancer Res. |volume=45 |issue= 11 Pt 1 |pages= 5405-12 |year= 1985 |pmid= 3931905 |doi=  }}
}}
}}
{{refend}}
{{refend}}


{{Nucleotide metabolism enzymes}}


{{Nucleotide metabolism enzymes}}
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Latest revision as of 09:50, 17 September 2017

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Identifiers
Aliases
External IDsGeneCards: [1]
Orthologs
SpeciesHumanMouse
Entrez

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PubMed searchn/an/a
Wikidata
View/Edit Human

Beta-ureidopropionase is an enzyme that in humans is encoded by the UPB1 gene.[1][2]

This gene encodes a protein that belongs to the CN hydrolase family. Beta-ureidopropionase catalyzes the last step in the pyrimidine degradation pathway. The pyrimidine bases uracil and thymine are degraded via the consecutive action of dihydropyrimidine dehydrogenase (DHPDH), dihydropyrimidinase (DHP) and beta-ureidopropionase (UP) to beta-alanine and beta-aminoisobutyric acid, respectively. UP deficiencies are associated with N-carbamyl-beta-amino aciduria and may lead to abnormalities in neurological activity.[2]

Interactive pathway map

Click on genes, proteins and metabolites below to link to respective articles.[§ 1]

[[File:
<imagemap> Image:FluoropyrimidineActivity_WP1601.png
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<imagemap> Image:FluoropyrimidineActivity_WP1601.png
|{{{bSize}}}px|alt=Fluorouracil (5-FU) Activity edit]]
Fluorouracil (5-FU) Activity edit
  1. ↑ The interactive pathway map can be edited at WikiPathways: "FluoropyrimidineActivity_WP1601".

References

  1. ↑ Vreken P, van Kuilenburg AB, Hamajima N, Meinsma R, van Lenthe H, Gohlich-Ratmann G, Assmann BE, Wevers RA, van Gennip AH (Dec 1999). "cDNA cloning, genomic structure and chromosomal localization of the human BUP-1 gene encoding beta-ureidopropionase". Biochim Biophys Acta. 1447 (2–3): 251–7. doi:10.1016/s0167-4781(99)00182-7. PMID 10542323.
  2. ↑ 2.0 2.1 "Entrez Gene: UPB1 ureidopropionase, beta".

Further reading



Retrieved from "https://www.wikidoc.org/index.php?title=UPB1&oldid=1420135"