Isobutyrylglycine (BioDeep_00000017902)

   

human metabolite Endogenous blood metabolite


代谢物信息卡片


2-(2-methylpropanamido)acetic acid

化学式: C6H11NO3 (145.0738896)
中文名称: 异丁酰基甘氨酸, N-异丁酰基甘氨酸
谱图信息: 最多检出来源 Homo sapiens(feces) 0.11%

分子结构信息

SMILES: CC(C)C(=O)NCC(=O)O
InChI: InChI=1S/C6H11NO3/c1-4(2)6(10)7-3-5(8)9/h4H,3H2,1-2H3,(H,7,10)(H,8,9)

描述信息

Isobutyrylglycine is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase (EC 2.3.1.13) which is an enzyme that catalyzes the chemical reaction: acyl-CoA + glycine < -- > CoA + N-acylglycine. Isobutyrylglycine is identified in large amount in urine of patients with isobutyryl-CoA dehydrogenase deficiency. Isobutyryl-CoA dehydrogenase deficiency is a disorder caused by the deficiency of isobutyryl-CoA dehydrogenase that is involved in the catabolism of the branched-chain amino acid valine (PMID 15505379). Moreover, Isobutyrylglycine is found to be associated with ethylmalonic encephalopathy and propionic acidemia, which are also inborn errors of metabolism. Isobutyrylglycine is a biomarker for the consumption of cheese.
Isobutyrylglycine is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase (EC 2.3.1.13) which is an enzyme that catalyzes the chemical reaction:

同义名列表

6 个代谢物同义名

2-(2-methylpropanamido)acetic acid; N-(1-Methylpropyl)glycine; N-Isobutanoylglycine; N-Isobutyrylglycine; Isobutanoylglycine; Isobutyrylglycine



数据库引用编号

11 个数据库交叉引用编号

分类词条

相关代谢途径

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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)

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2 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

  • Avalyn Stanislaus, Kevin Guo, Liang Li. Development of an isotope labeling ultra-high performance liquid chromatography mass spectrometric method for quantification of acylglycines in human urine. Analytica chimica acta. 2012 Oct; 750(?):161-72. doi: 10.1016/j.aca.2012.05.006. [PMID: 23062437]
  • Nagham George Abd Al-Ahad Sabbagha, Hsiao-Jung Kao, Chih-Fu Yang, Cheng-Chih Huang, Wei-De Lin, Fuu-Jen Tsai, Tzu-Ho Chen, Woan-Yuh Tarn, Jer-Yuarn Wu, Yuan-Tsong Chen. Alternative splicing in Acad8 resulting a mitochondrial defect and progressive hepatic steatosis in mice. Pediatric research. 2011 Jul; 70(1):31-6. doi: 10.1203/pdr.0b013e31821b89ee. [PMID: 21659959]
  • A Drousiotou, I DiMeo, R Mineri, Th Georgiou, G Stylianidou, V Tiranti. Ethylmalonic encephalopathy: application of improved biochemical and molecular diagnostic approaches. Clinical genetics. 2011 Apr; 79(4):385-90. doi: 10.1111/j.1399-0004.2010.01457.x. [PMID: 20528888]
  • 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]
  • Yan Liang, Li Liu, Hong Wei, Xiao-ping Luo, Mu-ti Wang. [Late-onset riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency (glutaric aciduria type II)]. Zhonghua er ke za zhi = Chinese journal of pediatrics. 2003 Dec; 41(12):916-20. doi: . [PMID: 14723814]
  • I L Bykov, E Shigematsu. [Effect of l-carnitine on the expression of glycine conjugates in rats in experimental B2-hypovitaminosis]. Voprosy pitaniia. 1997; ?(6):9-11. doi: NULL. [PMID: 9541994]
  • S Kølvraa, N Gregersen, E Christensen. In vivo studies on the metabolic derangement in a patient with D-glyceric acidaemia and hyperglycinaemia. Journal of inherited metabolic disease. 1984; 7(2):49-52. doi: 10.1007/bf01805799. [PMID: 6434823]
  • S Kølvraa, N Gregersen, N J Brandt. Excretion of short-chain N-acylglycines in the urine of a patient with D-glyceric acidemia. Clinica chimica acta; international journal of clinical chemistry. 1980 Sep; 106(2):215-21. doi: 10.1016/0009-8981(80)90174-6. [PMID: 7408214]