Sequence of BUP1_HUMAN
EC Number:3.5.1.6
EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
Reaction
3-ureidopropanoate + H2O = beta-alanine + CO2 + NH3
Other sequences found for EC No. 3.5.1.6
General information:
Sequence
0 MAGAEWKSLE ECLEKHLPLP DLQEVKRVLY GKELRKLDLP REAFEAASRE DFELQGYAFE
60 AAEEQLRRPR IVHVGLVQNR IPLPANAPVA EQVSALHRRI KAIVEVAAMC GVNIICFQEA
120 WTMPFAFCTR EKLPWTEFAE SAEDGPTTRF CQKLAKNHDM VVVSPILERD SEHGDVLWNT
180 AVVISNSGAV LGKTRKNHIP RVGDFNESTY YMEGNLGHPV FQTQFGRIAV NICYGRHHPL
240 NWLMYSINGA EIIFNPSATI GALSESLWPI EARNAAIANH CFTCAINRVG TEHFPNEFTS
300 GDGKKAHQDF GYFYGSSYVA APDSSRTPGL SRSRDGLLVA KLDLNLCQQV NDVWNFKMTG
360 RYEMYARELA EAVKSNYSPT IVKE
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Sequence Reference
Authors
Title
Journal
Volume
Pages
Year
PubMed ID
775534
Vreken P.,van Kuilenburg A.B.P.,Hamajima N.,Meinsma R.,van Lenthe H.,Goehlich-Ratmann G.,Assmann B.E.,Wevers R.A.,van Gennip A.H.
cDNA cloning, genomic structure and chromosomal localization of the human BUP-1 gene encoding beta-ureidopropionase.
Biochim. Biophys. Acta
1447
251-257
1999
775535
Sakamoto T.,Sakata S.F.,Matsuda K.,Horikawa Y.,Tamaki N.
Expression and properties of human liver beta-ureidopropionase.
J. Nutr. Sci. Vitaminol.
47
132-138
2001
775536
Collins J.E.,Wright C.L.,Edwards C.A.,Davis M.P.,Grinham J.A.,Cole C.G.,Goward M.E.,Aguado B.,Mallya M.,Mokrab Y.,Huckle E.J.,Beare D.M.,Dunham I.
A genome annotation-driven approach to cloning the human ORFeome.
Genome Biol.
5
0-0
2004
775538
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
Genome Res.
14
2121-2127
2004
775539
Van Kuilenburg A.B.,Van Lenthe H.,Van Gennip A.H.
A radiochemical assay for beta-ureidopropionase using radiolabeled N-carbamyl-beta-alanine obtained via hydrolysis of [2-(14)C]5, 6-dihydrouracil.
Anal. Biochem.
272
250-253
1999
775540
Bian Y.,Song C.,Cheng K.,Dong M.,Wang F.,Huang J.,Sun D.,Wang L.,Ye M.,Zou H.
An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
J. Proteomics
96
253-262
2014
775541
Maurer D.,Lohkamp B.,Krumpel M.,Widersten M.,Dobritzsch D.
Crystal structure and pH-dependent allosteric regulation of human beta-ureidopropionase, an enzyme involved in anticancer drug metabolism.
Biochem. J.
475
2395-2416
2018
775542
van Kuilenburg A.B.P.,Meinsma R.,Beke E.,Assmann B.,Ribes A.,Lorente I.,Busch R.,Mayatepek E.,Abeling N.G.G.M.,van Cruchten A.,Stroomer A.E.M.,van Lenthe H.,Zoetekouw L.,Kulik W.,Hoffmann G.F.,Voit T.,Wevers R.A.,Rutsch F.,van Gennip A.H.
Beta-ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities.
Hum. Mol. Genet.
13
2793-2801
2004
775543
van Kuilenburg A.B.,Dobritzsch D.,Meijer J.,Krumpel M.,Selim L.A.,Rashed M.S.,Assmann B.,Meinsma R.,Lohkamp B.,Ito T.,Abeling N.G.,Saito K.,Eto K.,Smitka M.,Engvall M.,Zhang C.,Xu W.,Zoetekouw L.,Hennekam R.C.
Beta-ureidopropionase deficiency: phenotype, genotype and protein structural consequences in 16 patients.
Biochim. Biophys. Acta
1822
1096-1108
2012
775544
Nakajima Y.,Meijer J.,Dobritzsch D.,Ito T.,Meinsma R.,Abeling N.G.,Roelofsen J.,Zoetekouw L.,Watanabe Y.,Tashiro K.,Lee T.,Takeshima Y.,Mitsubuchi H.,Yoneyama A.,Ohta K.,Eto K.,Saito K.,Kuhara T.,van Kuilenburg A.B.
Clinical, biochemical and molecular analysis of 13 Japanese patients with beta-ureidopropionase deficiency demonstrates high prevalence of the c.977G > A (p.R326Q) mutation [corrected].
J. Inherit. Metab. Dis.
37
801-812
2014
