2-hydroxy-3-methylvalerate (BioDeep_00000022911)

   

human metabolite Endogenous blood metabolite


代谢物信息卡片


(2R,3R)-2-Hydroxy-3-methyl-pentanoic acid

化学式: C6H12O3 (132.0786)
中文名称: 2-羟基-3-甲基戊酸
谱图信息: 最多检出来源 Homo sapiens(blood) 41.54%

Reviewed

Last reviewed on 2024-09-13.

Cite this Page

2-hydroxy-3-methylvalerate. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/2-hydroxy-3-methylvalerate (retrieved 2025-01-03) (BioDeep RN: BioDeep_00000022911). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: CCC(C)C(C(=O)O)O
InChI: InChI=1S/C6H12O3/c1-3-4(2)5(7)6(8)9/h4-5,7H,3H2,1-2H3,(H,8,9)

描述信息

2-Hydroxy-3-methylpentanoic acid or 2-hydroxy-3-methylvaleric acid (HMVA) is an organic acid generated by L-isoleucine metabolism. It is derived from the reduction of 2-Keto-3-methylvaleric acid (KMVA), possibly through the action of a lactate dehydrogenase (PMID: 1429566). There are 4 stereoisomers of HMVA (2S,3S-HMVA, 2R,3R-HMVA, 2S,3R-HMVA and 2R,3S-HMVA), of which the 2S,3S and 2S,3R derivatives are generally separable. HMVA is found in the urine and blood of normal individuals but in very elevated levels in patients with maple syrup urine disease (MSUD) (PMID: 1429566). Maple syrup urine disease (MSUD) is an inherited metabolic disease predominantly characterized by neurological dysfunction including psychomotor/delay/mental retardation. [HMDB]
2-Hydroxy-3-methylpentanoic acid or 2-hydroxy-3-methylvaleric acid (HMVA) is an organic acid generated by L-isoleucine metabolism. It is derived from the reduction of 2-Keto-3-methylvaleric acid (KMVA), possibly through the action of a lactate dehydrogenase (PMID: 1429566). There are 4 stereoisomers of HMVA (2S,3S-HMVA, 2R,3R-HMVA, 2S,3R-HMVA and 2R,3S-HMVA), of which the 2S,3S and 2S,3R derivatives are generally separable. HMVA is found in the urine and blood of normal individuals but in very elevated levels in patients with maple syrup urine disease (MSUD) (PMID: 1429566). Maple syrup urine disease (MSUD) is an inherited metabolic disease predominantly characterized by neurological dysfunction including psychomotor/delay/mental retardation.

同义名列表

18 个代谢物同义名

(2R,3R)-2-Hydroxy-3-methyl-pentanoic acid; (2R,3R)-2-Hydroxy-3-methylpentanoic acid; alpha-Hydroxy-beta-methylvaleric acid; (2R,3R)-2-Hydroxy-3-methyl-pentanoate; (2R,3R)-2-Hydroxy-3-methylpentanoate; alpha-Hydroxy-beta-methylvalerate; 2-hydroxy-3-methyl-pentanoic acid; 2-hydroxy-3-methylpentanoic acid; 2-Hydroxy-3-methyl-valeric acid; 2-Hydroxy-3-methylvaleric acid; a-Hydroxy-b-methylvaleric acid; 2-Hydroxy-3-methyl-pentanoate; 2-Hydroxy-3-methylpentanoate; 2-Hydroxy-3-methyl-valerate; a-Hydroxy-b-methylvalerate; 2-Hydroxy-3-methylvalerate; HMVA; 2-Hydroxy-3-methylpentanoic acid



数据库引用编号

10 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)

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。

亚细胞结构定位 关联基因列表


文献列表

  • Veronica von Saint Paul, Wei Zhang, Basem Kanawati, Birgit Geist, Theresa Faus-Kessler, Philippe Schmitt-Kopplin, Anton R Schäffner. The Arabidopsis glucosyltransferase UGT76B1 conjugates isoleucic acid and modulates plant defense and senescence. The Plant cell. 2011 Nov; 23(11):4124-45. doi: 10.1105/tpc.111.088443. [PMID: 22080599]
  • Till Beuerle, Claudine Theuring, Nico Klewer, Stefan Schulz, Thomas Hartmann. Absolute configuration of the creatonotines and callimorphines, two classes of arctiid-specific pyrrolizidine alkaloids. Insect biochemistry and molecular biology. 2007 Jan; 37(1):80-9. doi: 10.1016/j.ibmb.2006.10.005. [PMID: 17175448]
  • Vilson de Castro Vasques, Fabrício Brinco, Moacir Wajner. Intrahippocampal administration of the branched-chain alpha-hydroxy acids accumulating in maple syrup urine disease compromises rat performance in aversive and non-aversive behavioral tasks. Journal of the neurological sciences. 2005 May; 232(1-2):11-21. doi: 10.1016/j.jns.2004.12.015. [PMID: 15850577]
  • O A Mamer, M L Reimer. On the mechanisms of the formation of L-alloisoleucine and the 2-hydroxy-3-methylvaleric acid stereoisomers from L-isoleucine in maple syrup urine disease patients and in normal humans. The Journal of biological chemistry. 1992 Nov; 267(31):22141-7. doi: . [PMID: 1429566]
  • C Jakobs, L Sweetman, W L Nyhan. Hydroxy acid metabolites of branched-chain amino acids in amniotic fluid. Clinica chimica acta; international journal of clinical chemistry. 1984 Jul; 140(2):157-66. doi: 10.1016/0009-8981(84)90340-1. [PMID: 6467607]