3-hydroxy-2-ethylpropionate (BioDeep_00000027561)

   

human metabolite Endogenous blood metabolite


代谢物信息卡片


beta-Hydroxy-alpha-ethylpropionic acid

化学式: C5H10O3 (118.06299100000001)
中文名称: 2-(羟甲基)丁酸
谱图信息: 最多检出来源 Homo sapiens(blood) 2.27%

Reviewed

Last reviewed on 2024-09-13.

Cite this Page

3-hydroxy-2-ethylpropionate. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/3-hydroxy-2-ethylpropionate (retrieved 2024-11-05) (BioDeep RN: BioDeep_00000027561). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: CCC(CO)C(=O)O
InChI: InChI=1S/C5H10O3/c1-2-4(3-6)5(7)8/h4,6H,2-3H2,1H3,(H,7,8)

描述信息

Isolated excretion of 2-ethylhydracrylic acid (2-methylbutyrylglycine; 2-MBG) is the hallmark of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD), a defect in the proximal pathway of L-isoleucine oxidation (PMID: 15615815). Normal human urine contains small amounts (less than 4 mg/g of creatinine) of 2-ethylhydracrylic acid. Urinary excretion of 2-ethylhydracrylic acid is variably increased in defects of isoleucine oxidation at distal steps in the catabolic pathway (3-oxoacyl-CoA thiolase deficiency and methylmalonyl-CoA mutase deficiency) and is diminished when proximal steps of the oxidative pathway are blocked as in branched-chain oxo acid decarboxylase deficiency (maple syrup urine disease) (PMID: 1016232). 2-Ethylhydracrylic acid has been identified in the human placenta (PMID: 32033212).
Isolated excretion of 2-Ethylhydracrylic acid (2-methylbutyrylglycine; 2-MBG) is the hallmark of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD), a defect in the proximal pathway of L-isoleucine oxidation (PMID 15615815)

同义名列表

21 个代谢物同义名

beta-Hydroxy-alpha-ethylpropionic acid; 3-Hydroxy-propionic acid ethyl ester; beta-Hydroxy-alpha-ethylpropionate; Β-hydroxy-α-ethylpropionic acid; 3-Hydroxy-2-ethylpropionic acid; b-Hydroxy-a-ethylpropionic acid; 2-Ethyl-3-hydroxypropionic acid; 3-Hydroxy-2-ethylpropanoic acid; 2-(Hydroxymethyl)-butyric acid; 2-(hydroxymethyl)butanoic acid; 2-(Hydroxymethyl)butyric acid; 3-Hydroxy-2-ethylpropanoate; 2-Ethyl-3-hydroxypropionate; b-Hydroxy-a-ethylpropionate; 3-Hydroxy-2-ethylpropionate; Β-hydroxy-α-ethylpropionate; 2-(Hydroxymethyl)-butyrate; 2-(Hydroxymethyl)butyrate; 2-Ethylhydracrylic acid; 2-Ethyl-hydracrylate; 2-Ethylhydracrylate



数据库引用编号

7 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

0 个相关的代谢反应过程信息。

Reactome(0)

BioCyc(0)

WikiPathways(0)

Plant Reactome(0)

INOH(0)

PlantCyc(0)

COVID-19 Disease Map(0)

PathBank(0)

PharmGKB(0)

1 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

  • Michela Semeraro, Sara Boenzi, Rosalba Carrozzo, Daria Diodato, Diego Martinelli, Giorgia Olivieri, Giacomo Antonetti, Elisa Sacchetti, Giulio Catesini, Cristiano Rizzo, Carlo Dionisi-Vici. The urinary organic acids profile in single large-scale mitochondrial DNA deletion disorders. Clinica chimica acta; international journal of clinical chemistry. 2018 Jun; 481(?):156-160. doi: 10.1016/j.cca.2018.03.002. [PMID: 29534959]
  • Jana Konkoľová, Ján Chandoga, Juraj Kováčik, Marcel Repiský, Veronika Kramarová, Ivana Paučinová, Daniel Böhmer. Severe child form of primary hyperoxaluria type 2 - a case report revealing consequence of GRHPR deficiency on metabolism. BMC medical genetics. 2017 05; 18(1):59. doi: 10.1186/s12881-017-0421-8. [PMID: 28569194]
  • Robert O Ryan. Metabolic annotation of 2-ethylhydracrylic acid. Clinica chimica acta; international journal of clinical chemistry. 2015 Aug; 448(?):91-7. doi: 10.1016/j.cca.2015.06.012. [PMID: 26115894]
  • Saskia B Wortmann, Leo A Kluijtmans, Udo F H Engelke, Ron A Wevers, Eva Morava. The 3-methylglutaconic acidurias: what's new?. Journal of inherited metabolic disease. 2012 Jan; 35(1):13-22. doi: 10.1007/s10545-010-9210-7. [PMID: 20882351]
  • Feten Abbassi, Khaled Hani. In vitro antibacterial and antifungal activities of Rhus tripartitum used as antidiarrhoeal in Tunisian folk medicine. Natural product research. 2012; 26(23):2215-8. doi: 10.1080/14786419.2011.639072. [PMID: 22132736]
  • Makoto Takei, Yosuke Ando, Wataru Saitoh, Tomoe Tanimoto, Naoki Kiyosawa, Sunao Manabe, Atsushi Sanbuissho, Osamu Okazaki, Haruo Iwabuchi, Takashi Yamoto, Klaus-Peter Adam, James E Weiel, John A Ryals, Michael V Milburn, Lining Guo. Ethylene glycol monomethyl ether-induced toxicity is mediated through the inhibition of flavoprotein dehydrogenase enzyme family. Toxicological sciences : an official journal of the Society of Toxicology. 2010 Dec; 118(2):643-52. doi: 10.1093/toxsci/kfq211. [PMID: 20616209]
  • Judit García-Villoria, Aleix Navarro-Sastre, Carme Fons, Celia Pérez-Cerdá, Antonio Baldellou, Miguel Angel Fuentes-Castelló, Inmaculada González, Arturo Hernández-Gonzalez, Cristina Fernández, Jaume Campistol, Carina Delpiccolo, Nuria Cortés, Angel Messeguer, Paz Briones, Antonia Ribes. Study of patients and carriers with 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency: difficulties in the diagnosis. Clinical biochemistry. 2009 Jan; 42(1-2):27-33. doi: 10.1016/j.clinbiochem.2008.10.006. [PMID: 18996107]
  • Jörn Oliver Sass, Regina Ensenauer, Wulf Röschinger, Horst Reich, Ulrike Steuerwald, Oliver Schirrmacher, Katharina Engel, Johannes Häberle, Brage Storstein Andresen, André Mégarbané, Willy Lehnert, Johannes Zschocke. 2-Methylbutyryl-coenzyme A dehydrogenase deficiency: functional and molecular studies on a defect in isoleucine catabolism. Molecular genetics and metabolism. 2008 Jan; 93(1):30-5. doi: 10.1016/j.ymgme.2007.09.002. [PMID: 17945527]
  • Oivind J Kanavin, Berit Woldseth, Egil Jellum, Bjorn Tvedt, Brage S Andresen, Petter Stromme. 2-methylbutyryl-CoA dehydrogenase deficiency associated with autism and mental retardation: a case report. Journal of medical case reports. 2007 Sep; 1(?):98. doi: 10.1186/1752-1947-1-98. [PMID: 17883863]
  • Stanley H Korman, Brage S Andresen, Avraham Zeharia, Alisa Gutman, Avihu Boneh, James J Pitt. 2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation. Clinical chemistry. 2005 Mar; 51(3):610-7. doi: 10.1373/clinchem.2004.043265. [PMID: 15615815]
  • J P Petzer, J J Bergh, L J Mienie, N Castagnoli, C J Van der Schyf. Metabolic defects caused by 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) and by HPTP (the tetrahydropyridinyl analog of haloperidol), in rats. Life sciences. 2000 Apr; 66(20):1949-54. doi: 10.1016/s0024-3205(00)00520-8. [PMID: 10821119]
  • R J Pollitt, A Green, R Smith. Excessive excretion of beta-alanine and of 3-hydroxypropionic, R- and S-3-aminoisobutyric, R- and S-3-hydroxyisobutyric and S-2-(hydroxymethyl)butyric acids probably due to a defect in the metabolism of the corresponding malonic semialdehydes. Journal of inherited metabolic disease. 1985; 8(2):75-9. doi: 10.1007/bf01801669. [PMID: 3939535]
  • C Jakobs, L Sweetman, W L Nyhan. Hydroxy acid metabolites of branched-chain amino acids in amniotic fluid. Clinica chimica acta; international journal of clinical chemistry. 1984 Jul; 140(2):157-66. doi: 10.1016/0009-8981(84)90340-1. [PMID: 6467607]
  • O A Mamer, S S Tjoa, C R Scriver, G A Klassen. Demonstration of a new mammalian isoleucine catabolic pathway yielding an Rseries of metabolites. The Biochemical journal. 1976 Dec; 160(3):417-26. doi: 10.1042/bj1600417a. [PMID: 1016232]
  • O A Mamer, S S Tjoa. 2-Ethylhydracrylic acid: a newly described urinary organic acid. Clinica chimica acta; international journal of clinical chemistry. 1974 Sep; 55(2):199-204. doi: 10.1016/0009-8981(74)90295-2. [PMID: 4458986]