Ureidoisobutyric acid (BioDeep_00000019526)

Main id: BioDeep_00000005712

Secondary id: BioDeep_00001868825

human metabolite Endogenous blood metabolite natural product


代谢物信息卡片


(2S)-3-[(C-Hydroxycarbonimidoyl)amino]-2-methylpropanoate

化学式: C5H10N2O3 (146.0691)
中文名称:
谱图信息: 最多检出来源 Mus musculus(blood) 16.99%

分子结构信息

SMILES: CC(CNC(=N)O)C(=O)O
InChI: InChI=1S/C5H10N2O3/c1-3(4(8)9)2-7-5(6)10/h3H,2H2,1H3,(H,8,9)(H3,6,7,10)

描述信息

Ureidoisobutyric acid, also known as 3-ureidoisobutyrate or beta-UBA, belongs to the class of organic compounds known as ureas. Ureas are compounds containing two amine groups joined by a carbonyl (C=O) functional group. Ureidoisobutyric acid is an extremely weak basic (essentially neutral) compound (based on its pKa). Ureidoisobutyric acid exists in all living organisms, ranging from bacteria to humans. Within humans, ureidoisobutyric acid participates in a number of enzymatic reactions. In particular, ureidoisobutyric acid can be biosynthesized from dihydrothymine through its interaction with the enzyme dihydropyrimidinase. Outside of the human body, ureidoisobutyric acid has been detected, but not quantified in, several different foods, such as bread, squashberries, black elderberries, black crowberries, and climbing beans. This could make ureidoisobutyric acid a potential biomarker for the consumption of these foods. Ureidoisobutyric acid is increased in the urine of patients with beta-ureidopropionase (EC 3.5.1.6) deficiency (PMID: 12271438), a genetic disorder. Ureidoisobutyric acid can be used to predict a patients individual phenotypes of enzyme deficiencies in pyrimidine metabolism when associated with a risk for severe toxicity against the antineoplastic agent 5-fluorouracil (PMID: 12798197).

同义名列表

14 个代谢物同义名

(2S)-3-[(C-Hydroxycarbonimidoyl)amino]-2-methylpropanoate; 3-((Aminocarbonyl)amino)-2-methylpropanoic acid; 3-[(Aminocarbonyl)amino]-2-methylpropionic acid; 3-[(Aminocarbonyl)amino]-2-methylpropanoic acid; 3-((Aminocarbonyl)amino)-2-methylpropionic acid; (2S)-3-(Carbamoylamino)-2-methylpropanoic acid; (2S)-3-(Carbamoylamino)-2-methylpropionic acid; beta-Ureidoisobutyric acid; Β-ureidoisobutyric acid; Ureidoisobutyric acid; 3-Ureidoisobutyrate; Ureidoisobutyrate; beta-UBA; Β-uba



数据库引用编号

15 个数据库交叉引用编号

分类词条

相关代谢途径

Reactome(0)

BioCyc(0)

PlantCyc(0)

代谢反应

4 个相关的代谢反应过程信息。

Reactome(0)

BioCyc(0)

WikiPathways(0)

Plant Reactome(0)

INOH(0)

PlantCyc(4)

  • thymine degradation: 5,6-dihydrothymine + H2O ⟶ 3-(carbamoylamino)-2-methylpropanoate + H+
  • thymine degradation: 5,6-dihydrothymine + NADP+ ⟶ H+ + NADPH + thymine
  • thymine degradation: 5,6-dihydrothymine + H2O ⟶ 3-(carbamoylamino)-2-methylpropanoate + H+
  • thymine degradation: 3-(carbamoylamino)-2-methylpropanoate + H+ + H2O ⟶ (R)-3-amino-2-methylpropanoate + CO2 + ammonium

COVID-19 Disease Map(0)

PathBank(0)

PharmGKB(0)

15 个相关的物种来源信息

在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有：

  • PubMed: 来源于PubMed文献库中的文献信息，我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表，这个列表按照代谢物同时出现的文献数量降序排序，取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
  • NCBI Taxonomy: 通过文献数据挖掘，得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序，取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
  • Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
  • Chemical Reaction: 在化学反应过程中，存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。

点击图上的相关代谢物的名称，可以跳转到相关代谢物的信息页面。



文献列表

  • Péter Monostori, Glynis Klinke, Jana Hauke, Sylvia Richter, Jörgen Bierau, Sven F Garbade, Georg F Hoffmann, Claus-Dieter Langhans, Dorothea Haas, Jürgen G Okun. Extended diagnosis of purine and pyrimidine disorders from urine: LC MS/MS assay development and clinical validation. PloS one. 2019; 14(2):e0212458. doi: 10.1371/journal.pone.0212458. [PMID: 30817767]
  • John A Duley, Ming Ni, Catherine Shannon, Ross L Norris, Lesley Sheffield, Marion Harris, Andre B P van Kuilenburg, Scott Mead, Andrew Cameron, Nuala Helsby, Rani George, Bruce G Charles. Towards a test to predict 5-fluorouracil toxicity: Pharmacokinetic data for thymine and two sequential metabolites following oral thymine administration to healthy adult males. European journal of pharmaceutical sciences : official journal of the European Federation for Pharmaceutical Sciences. 2016 Jan; 81(?):36-41. doi: 10.1016/j.ejps.2015.10.001. [PMID: 26435217]
  • Ching-Wan Lam, Chun-Yiu Law, Ka-Fei Leung, Chi-Kong Lai, Sammy Pak-lam Chen, Bosco Chan, Kwok-Yin Chan, Yuet-ping Yuen, Chloe Miu Mak, Albert Yan-wo Chan. NMR-based urinalysis for rapid diagnosis of β-ureidopropionase deficiency in a patient with Dravet syndrome. Clinica chimica acta; international journal of clinical chemistry. 2015 Feb; 440(?):201-4. doi: 10.1016/j.cca.2014.10.030. [PMID: 25445412]
  • Ming Ni, John Duley, Rani George, Bruce Charles, Catherine Shannon, Ross McGeary, Ross Norris. Simultaneous determination of thymine and its sequential catabolites dihydrothymine and β-ureidoisobutyrate in human plasma and urine using liquid chromatography-tandem mass spectrometry with pharmacokinetic application. Journal of pharmaceutical and biomedical analysis. 2013 May; 78-79(?):129-35. doi: 10.1016/j.jpba.2013.01.038. [PMID: 23474813]
  • André B P van Kuilenburg, Doreen Dobritzsch, Judith Meijer, Michael Krumpel, Laila A Selim, Mohamed S Rashed, Birgit Assmann, Rutger Meinsma, Bernhard Lohkamp, Tetsuya Ito, Nico G G M Abeling, Kayoko Saito, Kaoru Eto, Martin Smitka, Martin Engvall, Chunhua Zhang, Wang Xu, Lida Zoetekouw, Raoul C M Hennekam. ß-ureidopropionase deficiency: phenotype, genotype and protein structural consequences in 16 patients. Biochimica et biophysica acta. 2012 Jul; 1822(7):1096-108. doi: 10.1016/j.bbadis.2012.04.001. [PMID: 22525402]
  • T Kuhara, M Ohse, Y Inoue, T Shinka. Five cases of beta-ureidopropionase deficiency detected by GC/MS analysis of urine metabolome. Journal of mass spectrometry : JMS. 2009 Feb; 44(2):214-21. doi: 10.1002/jms.1500. [PMID: 18853477]
  • André B P van Kuilenburg, Judith Meijer, Doreen Dobritzsch, Rutger Meinsma, Marinus Duran, Bernhard Lohkamp, Lida Zoetekouw, Nico G G M Abeling, Herman L G van Tinteren, Annet M Bosch. Clinical, biochemical and genetic findings in two siblings with a dihydropyrimidinase deficiency. Molecular genetics and metabolism. 2007 Jun; 91(2):157-64. doi: 10.1016/j.ymgme.2007.02.008. [PMID: 17383919]
  • André B P van Kuilenburg, Rutger Meinsma, Eva Beke, Birgit Assmann, Antonia Ribes, Isabel Lorente, Rebekka Busch, Ertan Mayatepek, Nico G G M Abeling, Arno van Cruchten, Alida E M Stroomer, Henk van Lenthe, Lida Zoetekouw, Willem Kulik, Georg F Hoffmann, Thomas Voit, Ron A Wevers, Frank Rutsch, Albert H van Gennip. beta-Ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities. Human molecular genetics. 2004 Nov; 13(22):2793-801. doi: 10.1093/hmg/ddh303. [PMID: 15385443]
  • Ute Hofmann, Matthias Schwab, Sonja Seefried, Claudia Marx, Ulrich M Zanger, Michel Eichelbaum, Thomas E Mürdter. Sensitive method for the quantification of urinary pyrimidine metabolites in healthy adults by gas chromatography-tandem mass spectrometry. Journal of chromatography. B, Analytical technologies in the biomedical and life sciences. 2003 Jul; 791(1-2):371-80. doi: 10.1016/s1570-0232(03)00251-4. [PMID: 12798197]
  • Morimasa Ohse, Masafumi Matsuo, Akihito Ishida, Tomiko Kuhara. Screening and diagnosis of beta-ureidopropionase deficiency by gas chromatographic/mass spectrometric analysis of urine. Journal of mass spectrometry : JMS. 2002 Sep; 37(9):954-62. doi: 10.1002/jms.354. [PMID: 12271438]
  • S H Moolenaar, G Göhlich-Ratmann, U F Engelke, M Spraul, E Humpfer, P Dvortsak, T Voit, G F Hoffmann, C Bräutigam, A B van Kuilenburg, A van Gennip, P Vreken, R A Wevers. beta-Ureidopropionase deficiency: a novel inborn error of metabolism discovered using NMR spectroscopy on urine. Magnetic resonance in medicine. 2001 Nov; 46(5):1014-7. doi: 10.1002/mrm.1289. [PMID: 11675655]
  • T A Walsh, S B Green, I M Larrinua, P R Schmitzer. Characterization of plant beta-ureidopropionase and functional overexpression in Escherichia coli. Plant physiology. 2001 Feb; 125(2):1001-11. doi: 10.1104/pp.125.2.1001. [PMID: 11161056]
  • S T TAKATS, R M SMELLIE. Thymidine degradation products in plant tissues labeled with tritiated thymidine. The Journal of cell biology. 1963 Apr; 17(?):59-66. doi: 10.1083/jcb.17.1.59. [PMID: 13984611]