3-Methylcrotonylglycine (BioDeep_00000001239)

 

Secondary id: BioDeep_00000405278

human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite


代谢物信息卡片


N-(3-Methyl-1-oxo-2-butenyl)-glycine

化学式: C7H11NO3 (157.0738896)
中文名称: 3-甲基巴豆酰甘氨酸, N -(3 -甲基- 1 -氧代- 2 -丁烯基)氨基乙酸
谱图信息: 最多检出来源 Chinese Herbal Medicine(otcml) 2.29%

Reviewed

Last reviewed on 2024-09-13.

Cite this Page

3-Methylcrotonylglycine. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/3-methylcrotonylglycine (retrieved 2024-11-09) (BioDeep RN: BioDeep_00000001239). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: CC(=CC(=O)NCC(=O)O)C
InChI: InChI=1S/C7H11NO3/c1-5(2)3-6(9)8-4-7(10)11/h3H,4H2,1-2H3,(H,8,9)(H,10,11)

描述信息

3-Methylcrotonylglycine 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. 3-Methylcrotonylglycine is a normal amino acid metabolite found in urine. Increased levels of this metabolite are found in patients suffering from leucine catabolic disorders, such as 3-methylcrotonyl-CoA carboxylase deficiency. 3-Methylcrotonylglycine is often considered to be a diagnostic marker of organic acidemias (PMID 11170888). Moreover, 3-methylcrotonylglycine is found to be associated with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency and propionic acidemia, which are also inborn errors of metabolism.
3-Methylcrotonylglycine 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:
3-Methylcrotonylglycine is an acyl glycine, a normal amino acid metabolite found in urine.

同义名列表

10 个代谢物同义名

N-(3-Methyl-1-oxo-2-butenyl)-glycine; 2-(3-methylbut-2-enamido)acetic acid; (3-methylbut-2-enamido)acetic acid; beta-Methylcrotonylglycine; 3-Methylcrotonyl glycine; 3-Methylcrotonyl-glycine; 3-Methylcrotonylglycine; Β-methylcrotonylglycine; b-Methylcrotonylglycine; 3-Methylcrotonylglycin



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

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)

3 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

  • Francesca Santarelli, Michela Cassanello, Ausilia Enea, Francesca Poma, Valentina D'Onofrio, Giovanna Guala, Giangiacomo Garrone, Paola Puccinelli, Ubaldo Caruso, Francesco Porta, Marco Spada. A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency. Italian journal of pediatrics. 2013 May; 39(?):33. doi: 10.1186/1824-7288-39-33. [PMID: 23705938]
  • 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]
  • Alana Pimentel Moura, César Augusto João Ribeiro, Ângela Zanatta, Estela Natacha Brandt Busanello, Anelise Miotti Tonin, Moacir Wajner. 3-Methylcrotonylglycine disrupts mitochondrial energy homeostasis and inhibits synaptic Na(+),K (+)-ATPase activity in brain of young rats. Cellular and molecular neurobiology. 2012 Mar; 32(2):297-307. doi: 10.1007/s10571-011-9761-7. [PMID: 21993987]
  • Ali Al Kaissi, Sabine Scholl-Buergi, Rainer Biedermann, Kathrin Maurer, Jochen G Hofstaetter, Klaus Klaushofer, Franz Grill. The diagnosis and management of patients with idiopathic osteolysis. Pediatric rheumatology online journal. 2011 Oct; 9(?):31. doi: 10.1186/1546-0096-9-31. [PMID: 21995273]
  • Konstantinos A Kouremenos, James Pitt, Philip J Marriott. Metabolic profiling of infant urine using comprehensive two-dimensional gas chromatography: Application to the diagnosis of organic acidurias and biomarker discovery. Journal of chromatography. A. 2010 Jan; 1217(1):104-11. doi: 10.1016/j.chroma.2009.10.033. [PMID: 19896671]
  • Guilhian Leipnitz, Bianca Seminotti, Carolina G Fernandes, Alexandre U Amaral, Ana Paula Beskow, Lucila de B da Silva, Angela Zanatta, César A J Ribeiro, Carmen R Vargas, Moacir Wajner. Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency as compared to liver. International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience. 2009 Jun; 27(4):351-6. doi: 10.1016/j.ijdevneu.2009.03.001. [PMID: 19460629]
  • F Tuba Eminoglu, Aysima A Ozcelik, Ilyas Okur, Leyla Tumer, Gursel Biberoglu, Ercan Demir, Alev Hasanoglu, Matthias R Baumgartner. 3-Methylcrotonyl-CoA carboxylase deficiency: phenotypic variability in a family. Journal of child neurology. 2009 Apr; 24(4):478-81. doi: 10.1177/0883073808324536. [PMID: 19339287]
  • Saskia Koene, Leo A J Kluijtmans, Ron Wevers, Donald Mock, Marcel Pasch, Eva Morava. Biotin-sensitive 3-methylcrotonylglycinuria in a patient with severe growth delay, ectodermal abnormalities, neonatal progeroid appearance, and developmental delay. Clinical dysmorphology. 2008 Jul; 17(3):195-198. doi: 10.1097/mcd.0b013e3282fe99c4. [PMID: 18541968]
  • Lynne A Wolfe, David N Finegold, Jerry Vockley, Nicole Walters, Celine Chambaz, Terttu Suormala, Hans Georg Koch, Dietrich Matern, Bruce A Barshop, Lorna J Cropcho, Matthias R Baumgartner, K Michael Gibson. Potential misdiagnosis of 3-methylcrotonyl-coenzyme A carboxylase deficiency associated with absent or trace urinary 3-methylcrotonylglycine. Pediatrics. 2007 Nov; 120(5):e1335-40. doi: 10.1542/peds.2007-0674. [PMID: 17908719]
  • L Pinto, P Zen, R Rosa, G Paskulin, A Perla, L Barea, M R Baumgartner, M F Dantas, B Fowler, R Giugliani, C Vargas, M Wajner, C Graziadio. Isolated 3-methylcrotonyl-coenzyme A carboxylase deficiency in a child with metabolic stroke. Journal of inherited metabolic disease. 2006 Feb; 29(1):205-6. doi: 10.1007/s10545-006-0188-0. [PMID: 16601894]
  • Wataru Fujimoto, Makoto Inaoki, Toru Fukui, Yoshito Inoue, Tomiko Kuhara. Biotin deficiency in an infant fed with amino acid formula. The Journal of dermatology. 2005 Apr; 32(4):256-61. doi: 10.1111/j.1346-8138.2005.tb00758.x. [PMID: 15863846]
  • T Baykal, G Huner Gokcay, Z Ince, M F Dantas, B Fowler, M R Baumgartner, F Demir, G Can, M Demirkol. Consanguineous 3-methylcrotonyl-CoA carboxylase deficiency: early-onset necrotizing encephalopathy with lethal outcome. Journal of inherited metabolic disease. 2005; 28(2):229-33. doi: 10.1007/s10545-005-4559-8. [PMID: 15877210]
  • Matthias R Baumgartner, M Fernanda Dantas, Terttu Suormala, Shlomo Almashanu, Cecilia Giunta, Dolores Friebel, Boris Gebhardt, Brian Fowler, Georg F Hoffmann, E Regula Baumgartner, David Valle. Isolated 3-methylcrotonyl-CoA carboxylase deficiency: evidence for an allele-specific dominant negative effect and responsiveness to biotin therapy. American journal of human genetics. 2004 Nov; 75(5):790-800. doi: 10.1086/425181. [PMID: 15359379]
  • D D Koeberl, D S Millington, W E Smith, S D Weavil, J Muenzer, S E McCandless, P S Kishnani, M T McDonald, S Chaing, A Boney, E Moore, D M Frazier. Evaluation of 3-methylcrotonyl-CoA carboxylase deficiency detected by tandem mass spectrometry newborn screening. Journal of inherited metabolic disease. 2003; 26(1):25-35. doi: 10.1023/a:1024015227863. [PMID: 12872837]
  • Donald M Mock, Nell I Mock. Lymphocyte propionyl-CoA carboxylase is an early and sensitive indicator of biotin deficiency in rats, but urinary excretion of 3-hydroxypropionic acid is not. The Journal of nutrition. 2002 Jul; 132(7):1945-50. doi: 10.1093/jn/132.7.1945. [PMID: 12097674]
  • H Ogier de Baulny, J M Saudubray. Branched-chain organic acidurias. Seminars in neonatology : SN. 2002 Feb; 7(1):65-74. doi: 10.1053/siny.2001.0087. [PMID: 12069539]
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  • W Röschinger, D S Millington, D A Gage, Z H Huang, T Iwamoto, S Yano, S Packman, K Johnston, S A Berry, L Sweetman. 3-Hydroxyisovalerylcarnitine in patients with deficiency of 3-methylcrotonyl CoA carboxylase. Clinica chimica acta; international journal of clinical chemistry. 1995 Aug; 240(1):35-51. doi: 10.1016/0009-8981(95)06126-2. [PMID: 8582058]
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  • J Mourmans, J Bakkeren, J de Jong, R Wevers, O P van Diggelen, T Suormala, R Baumgartner, U Wendel. Isolated (biotin-resistant) 3-methylcrotonyl-CoA carboxylase deficiency: four sibs devoid of pathology. Journal of inherited metabolic disease. 1995; 18(5):643-5. doi: 10.1007/bf02436014. [PMID: 8598650]
  • M Tuchman, S A Berry, L P Thuy, W L Nyhan. Partial methylcrotonyl-coenzyme A carboxylase deficiency in an infant with failure to thrive, gastrointestinal dysfunction, and hypertonia. Pediatrics. 1993 Mar; 91(3):664-6. doi: NULL. [PMID: 8441580]
  • D M Mock, N I Mock, S Weintraub. Abnormal organic aciduria in biotin deficiency: the rat is similar to the human. The Journal of laboratory and clinical medicine. 1988 Aug; 112(2):240-7. doi: . [PMID: 3397627]
  • E Holme, C E Jacobson, B Kristiansson. Biotin-responsive multiple carboxylase deficiency in an 8-year-old boy with normal serum biotinidase and fibroblast holocarboxylase-synthetase activities. Journal of inherited metabolic disease. 1988; 11(3):270-6. doi: 10.1007/bf01800369. [PMID: 3148068]
  • C Dionisi-Vici, C Bachmann, M C Graziani, G Sabetta. Laryngeal stridor as a leading symptom in a biotinidase-deficient patient. Journal of inherited metabolic disease. 1988; 11(3):312-3. doi: 10.1007/bf01800381. [PMID: 3148078]
  • D M Mock, D L Baswell, H Baker, R T Holman, L Sweetman. Biotin deficiency complicating parenteral alimentation: diagnosis, metabolic repercussions, and treatment. The Journal of pediatrics. 1985 May; 106(5):762-9. doi: 10.1016/s0022-3476(85)80350-4. [PMID: 3923177]
  • J Greter, E Holme, S Lindstedt, M Koivikko. Biotin-responsive 3-methylcrotonylglycinuria with biotinidase deficiency. Journal of inherited metabolic disease. 1985; 8 Suppl 2(?):103-4. doi: 10.1007/bf01811479. [PMID: 3930850]
  • L Sweetman, L Surh, H Baker, R M Peterson, W L Nyhan. Clinical and metabolic abnormalities in a boy with dietary deficiency of biotin. Pediatrics. 1981 Oct; 68(4):553-8. doi: . [PMID: 7322688]
  • K Bartlett, H Ng, J V Leonard. A combined defect of three mitochondrial carboxylases presenting as biotin-responsive 3-methylcrotonyl glycinuria and 3-hydroxyisovaleric aciduria. Clinica chimica acta; international journal of clinical chemistry. 1980 Jan; 100(2):183-6. doi: 10.1016/0009-8981(80)90081-9. [PMID: 6766095]
  • P Purkiss, R A Chalmers, O Borud. Combined iminoglycinuria and cystine- and dibasic aminoaciduria in patients with propionic acidaemia and 3-methylcrotonylglycinuria. Journal of inherited metabolic disease. 1980; 3(3):85-6. doi: 10.1007/bf02312533. [PMID: 6775144]
  • L Giuliani, G Carmignani, E Belgrano, P Puppo. Transcatheter arterial embolization in urological tumors: the use of isobutyl-2-cyanoacrylate. The Journal of urology. 1979 May; 121(5):630-4. doi: 10.1016/s0022-5347(17)56913-x. [PMID: 439260]
  • S J Wysocki, R Hähnel. 3-Methylcrotonylglycine excretion in 3-hydroxy-3-methylglutaric aciduria. Clinica chimica acta; international journal of clinical chemistry. 1978 May; 86(1):101-8. doi: 10.1016/0009-8981(78)90464-3. [PMID: 657530]