Isovalerylglycine (BioDeep_00000017903)
Main id: BioDeep_00000405332
human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite Chemicals and Drugs
代谢物信息卡片
化学式: C7H13NO3 (159.0895388)
中文名称: N-异戊酰氨基乙酸
谱图信息:
最多检出来源 () 0%
分子结构信息
SMILES: CC(C)CC(=O)NCC(=O)O
InChI: InChI=1S/C7H13NO3/c1-5(2)3-6(9)8-4-7(10)11/h5H,3-4H2,1-2H3,(H,8,9)(H,10,11)
描述信息
Isovalerylglycine (IVG) 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. Isovalerylglycine is a byproduct of the catabolism of the aminoacid leucine. Accumulation of isovalerylglycine occurs in Isovaleric Acidemia (IVA). IVA (OMIM/ McKusick 243500) is an autosomal recessive disorder caused by mutations in the isovaleryl-CoA dehydrogenase (EC 1.3.99.10) gene. The deficiency of this enzyme in the metabolism of leucine leads to the accumulation of a series of isovaleryl-CoA metabolites, such as isovalerylglycine. It is very important to caution for false positive results when screening for isovaleric acidemia by tandem mass spectrometry based on dried blood-spot levels of C5-acylcarnitines, including isovalerylcarnitine and its isomer, pivaloylcarnitine; pivaloylcarnitine is derived from pivalate-generating antibiotics, and has caused many false-positive results (PMID: 17850781). Isovalerylglycine is a biomarker for the consumption of cheese.
Isovalerylglycine 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:
N-Isovaleroylglycine is an acyl glycine and could be used as a biomarker for the predispositon for weight gain and obesity.
同义名列表
12 个代谢物同义名
2-(3-Methylbutanoylamino)ethanoic acid; [(3-Methylbutanoyl)amino]acetic acid; 2-(3-Methylbutanoylamino)ethanoate; 2-(3-methylbutanamido)acetic acid; [(3-Methylbutanoyl)amino]acetate; 3-Methylbutyrylglycine; N-Isopentanoylglycine; N-Isovaleroylglycine; N-Isovaleryl-glycine; Isopentanoylglycine; N-Isovalerylglycine; Isovalerylglycine
数据库引用编号
9 个数据库交叉引用编号
- ChEBI: CHEBI:70984
- PubChem: 546304
- HMDB: HMDB0000678
- foodb: FDB022177
- chemspider: 475516
- CAS: 16284-60-9
- PMhub: MS000000990
- RefMet: Isovalerylglycine
- medchemexpress: HY-W015464
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)
7 个相关的物种来源信息
- 9606 Homo sapiens: 10.1007/S11306-016-1051-4
- 654 Aeromonas veronii: 10.3389/FCIMB.2020.00044
- 7227 Drosophila melanogaster: 10.1038/S41467-019-11933-Z
- 9606 Homo sapiens: 10.1007/S11306-016-1051-4
- 654 Aeromonas veronii: 10.3389/FCIMB.2020.00044
- 7227 Drosophila melanogaster: 10.1038/S41467-019-11933-Z
- 9606 Homo sapiens: -
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
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Journal of human genetics.
2017 Mar; 62(3):355-360. doi:
10.1038/jhg.2016.144. [PMID: 27904153] - 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.
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Toxicological sciences : an official journal of the Society of Toxicology.
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Pediatrics international : official journal of the Japan Pediatric Society.
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Cellular and molecular neurobiology.
2007 Jun; 27(4):529-40. doi:
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