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 (157.0739)
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 Homo sapiens(otcml) 30.14%

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 2024-12-23) (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。

亚细胞结构定位 关联基因列表
Cytoplasm 5 ACADM, CAT, MDM2, PC, WT1
Nucleus 3 ACADM, MDM2, WT1
cytosol 8 CAT, GSR, HMGCL, MCCC1, MCCC2, MDM2, PC, WT1
mitochondrial membrane 1 ACADM
nucleoplasm 3 ATP2B1, MDM2, WT1
Cell membrane 1 ATP2B1
Multi-pass membrane protein 1 ATP2B1
Synapse 1 ATP2B1
glutamatergic synapse 2 ATP2B1, MDM2
presynaptic membrane 1 ATP2B1
plasma membrane 2 ATP2B1, MDM2
synaptic vesicle membrane 1 ATP2B1
Membrane 2 ATP2B1, CAT
axon 1 ACADM
basolateral plasma membrane 1 ATP2B1
extracellular exosome 4 ATP2B1, BTD, CAT, GSR
extracellular space 1 BTD
mitochondrion 7 ACADM, CAT, GSR, HMGCL, MCCC1, MCCC2, PC
protein-containing complex 3 CAT, HMGCL, MDM2
intracellular membrane-bounded organelle 2 ATP2B1, CAT
postsynaptic density 1 MDM2
extracellular region 2 BTD, CAT
Mitochondrion matrix 5 ACADM, HMGCL, MCCC1, MCCC2, PC
mitochondrial matrix 8 ACADM, BTD, CAT, GSR, HMGCL, MCCC1, MCCC2, PC
Cytoplasmic vesicle, secretory vesicle, synaptic vesicle membrane 1 ATP2B1
external side of plasma membrane 1 GSR
nucleolus 2 MDM2, WT1
focal adhesion 1 CAT
Peroxisome 2 CAT, HMGCL
Peroxisome matrix 1 CAT
peroxisomal matrix 2 CAT, HMGCL
peroxisomal membrane 1 CAT
lateral plasma membrane 1 ATP2B1
nuclear speck 1 WT1
cell projection 1 ATP2B1
Secreted, extracellular space 1 BTD
Nucleus, nucleolus 1 MDM2
Basolateral cell membrane 1 ATP2B1
Nucleus, nucleoplasm 1 MDM2
Nucleus speckle 1 WT1
Presynaptic cell membrane 1 ATP2B1
ficolin-1-rich granule lumen 1 CAT
secretory granule lumen 1 CAT
transcription repressor complex 1 MDM2
endocytic vesicle membrane 1 MDM2
immunological synapse 1 ATP2B1
catalase complex 1 CAT
photoreceptor ribbon synapse 1 ATP2B1
[Isoform 1]: Nucleus speckle 1 WT1
[Isoform 4]: Nucleus, nucleoplasm 1 WT1
3-methylcrotonyl-CoA carboxylase complex, mitochondrial 2 MCCC1, MCCC2
methylcrotonoyl-CoA carboxylase complex 2 MCCC1, MCCC2


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]
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  • 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]
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  • 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]
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  • 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]
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  • 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]
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