Isovalerylalanine (BioDeep_00000027644)

   

human metabolite Endogenous


代谢物信息卡片


(2S)-2-(3-Methylbutanamido)propanoic acid

化学式: C8H15NO3 (173.105188)
中文名称:
谱图信息: 最多检出来源 () 0%

分子结构信息

SMILES: CC(C)CC(=O)NC(C)C(=O)O
InChI: InChI=1S/C8H15NO3/c1-5(2)4-7(10)9-6(3)8(11)12/h5-6H,4H2,1-3H3,(H,9,10)(H,11,12)/t6-/m0/s1

描述信息

Isovalerylalanine, also known as isopentanoylalanine, belongs to the class of organic compounds known as N-acyl-L-alpha-amino acids. These are N-acylated alpha amino acids which have the L-configuration of the alpha-carbon atom. Isovalerylalanine results from the formal condensation of the carboxy group of isovaleric acid with the amino group of L-alanine. Isovalerylalanine is one of the constituents of the organic acid profile in isovaleric acidemia, it is believed to be formed by the action of the enzyme glycine N-acylase on alanine, using isovaleryl-CoA as a substrate (PMID: 6197208). Isovaleric acidemia was the first organic acid acidemia to be documented in humans. It is an autosomal recessive inborn error in the metabolism of leucine, caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase (IVD) that results in the accumulation isovaleryl-CoA derivatives (PMID: 16602101). The excess of isovaleryl-CoA in plasma allows for the formation of condensation derivatives, such as isovalerylalanine which makes it one of the biomarkers for this condition.
Isovalerylalanine is a metabolite

同义名列表

8 个代谢物同义名

(2S)-2-(3-Methylbutanamido)propanoic acid; (2S)-2-(3-Methylbutanamido)propanoate; N-Isopentanoyl-L-alanine; N-Isovaleryl-L-alanine; N-Isopentanoylalanine; Isopentanoylalanine; N-Isovalerylalanine; Isovalerylalanine



数据库引用编号

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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。

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1 个相关的物种来源信息

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  • 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。
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  • 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。

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文献列表

  • W Lehnert. N-Isovalerylalanine and N-isovalerylsarcosine: two new minor metabolites in isovaleric acidemia. Clinica chimica acta; international journal of clinical chemistry. 1983 Oct; 134(1-2):207-12. doi: 10.1016/0009-8981(83)90198-5. [PMID: 6197208]