2-Hydroxy-3-methylbutyric acid (BioDeep_00000017754)

 

Secondary id: BioDeep_00000629016

human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite


代谢物信息卡片


2-Hydroxyisovaleric acid, monosodium salt, (S)-isomer

化学式: C5H10O3 (118.063)
中文名称: 2-羟基-3-甲基丁酸
谱图信息: 最多检出来源 Homo sapiens(not specific) 8.11%

Reviewed

Last reviewed on 2024-09-13.

Cite this Page

2-Hydroxy-3-methylbutyric acid. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/2-hydroxy-3-methylbutyric_acid (retrieved 2025-03-12) (BioDeep RN: BioDeep_00000017754). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: CC(C)C(C(=O)O)O
InChI: InChI=1S/C5H10O3/c1-3(2)4(6)5(7)8/h3-4,6H,1-2H3,(H,7,8)

描述信息

2-Hydroxy-3-methylbutyric acid (also known as 2-hydroxyisovaleric acid) is a metabolite found in the urine of patients with phenylketonuria (PMID: 7978272), methylmalonic acidemia, propionic acidemia, 3-ketothiolase deficiency, isovaleric acidemia, 3-methylcrotonylglycemia, 3-hydroxy-3-methylglutaric acidemia, multiple carboxylase deficiency, glutaric aciduria, ornithine transcarbamylase deficiency, glyceroluria, tyrosinemia type I, galactosemia, and maple syrup urine disease (PMID: 11048741). 2-Hydroxyisovaleric acid has also been identified in the urine of patients with lactic acidosis and ketoacidosis (PMID: 884872), and in the urine of severely asphyxiated babies (PMID: 1610944). 2-Hydroxyisovaleric acid originates mainly from ketogenesis and from the metabolism of valine, leucine, and isoleucine (PMID: 6434570). 2-Hydroxy-3-methylbutyric acid has been identified in the human placenta (PMID: 32033212).
2-Hydroxy-3-methylbutyric acid is a metabolite found in the urine of patients with Phenylketonuria (PMID 7978272), Methylmalonic acidemia, Propionic acidemia, 3-Ketothiolase deficiency, Isovaleric acidemia, 3-Methylcrotonylglycemia, 3-Hydroxy-3-methylglutaric acidemia, Multiple carboxylase deficiency, Glutaric aciduria, Ornithine transcarbamylase deficiency, glyceroluria, Tyrosinemia type 1, Galactosemia, and Maple syrup urine disease (PMID 11048741) [HMDB]
2-Hydroxy-3-methylbutanoic acid is a close structure analogue of GHB, which is a naturally occurring neurotransmitter and a psychoactive agent.

同义名列表

33 个代谢物同义名

2-Hydroxyisovaleric acid, monosodium salt, (S)-isomer; 2-Hydroxyisovaleric acid, calcium (2:1) salt; 2-Hydroxyisovaleric acid, (S)-isomer; 2-Hydroxyisovaleric acid, (R)-isomer; DL-2-Hydroxy-3-methylbutanoic acid; DL-alpha-Hydroxyisovaleric acid; 2-hydroxy-3-methylbutanoic acid; 2-hydroxy-3-methyl-butyric acid; 2-Hydroxy-3-methylbutyric acid; DL-2-Hydroxy-3-methylbutanoate; 3-Methyl-2-hydroxybutyric acid; alpha-Hydroxyisovaleric acid; DL-a-Hydroxyisovaleric acid; 2-Hydroxy-3-methylbutanoate; DL-2-Hydroxyisovaleric acid; DL-alpha-Hydroxyisovalerate; 2-Hydroxy-3-methylbutyrate; 3-Methyl-2-hydroxybutyrate; 2-Hydroxyisopentanoic acid; Α-hydroxyisovaleric acid; 2-Hydroxyisovaleric acid; a-Hydroxyisovaleric acid; alpha-Hydroxyisovalerate; DL-2-Hydroxyisovalerate; DL-a-Hydroxyisovalerate; 2-Hydroxyisopentanoate; 2-Hydroxyisovalerate; a-Hydroxyisovalerate; 2-Oxyisovaleric acid; Α-hydroxyisovalerate; 2-Oxyisovalerate; 2-Hydroxy-3-methylbutyric acid; 2-Hydroxy-3-methylbutyric acid



数据库引用编号

13 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)

5 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

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  • Tao Sun, Ying Wu, Xueping Wu, Haifeng Ma. Metabolomic profiles investigation on athletes' urine 35 minutes after an 800-meter race. The Journal of sports medicine and physical fitness. 2017 Jun; 57(6):839-849. doi: 10.23736/s0022-4707.17.06254-5. [PMID: 26699119]
  • Hamza Mostafa, Arwa M Amin, Chin-Hoe Teh, Vikneswaran Murugaiyah, Nor Hayati Arif, Baharudin Ibrahim. Metabolic phenotyping of urine for discriminating alcohol-dependent from social drinkers and alcohol-naive subjects. Drug and alcohol dependence. 2016 Dec; 169(?):80-84. doi: 10.1016/j.drugalcdep.2016.10.016. [PMID: 27788404]
  • Su-Qing Qu, Li-Cai Yang, Zuo Luan, Kan Du, Hui Yang. [Acute encephalopathy due to late-onset maple syrup urine disease in a school boy]. Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics. 2012 Mar; 14(3):161-4. doi: . [PMID: 22433398]
  • Ram Chandra, Ram Naresh Bharagava, Atya Kapley, Hemant J Purohit. Bacterial diversity, organic pollutants and their metabolites in two aeration lagoons of common effluent treatment plant (CETP) during the degradation and detoxification of tannery wastewater. Bioresource technology. 2011 Feb; 102(3):2333-41. doi: 10.1016/j.biortech.2010.10.087. [PMID: 21075615]
  • Chunfeng Lu, Yimei Wang, Zhiguo Sheng, Gang Liu, Ze Fu, Jing Zhao, Jun Zhao, Xianzhong Yan, Benzhan Zhu, Shuangqing Peng. NMR-based metabonomic analysis of the hepatotoxicity induced by combined exposure to PCBs and TCDD in rats. Toxicology and applied pharmacology. 2010 Nov; 248(3):178-84. doi: 10.1016/j.taap.2010.07.020. [PMID: 20691717]
  • Alexandre Umpierrez Amaral, Guilhian Leipnitz, Carolina Gonçalves Fernandes, Bianca Seminotti, Patrícia Fernanda Schuck, Moacir Wajner. Alpha-ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain. Brain research. 2010 Apr; 1324(?):75-84. doi: 10.1016/j.brainres.2010.02.018. [PMID: 20153737]
  • Chang Shi, Chun-Qi Wu, An-Min Cao, He-Zhang Sheng, Xian-Zhong Yan, Ming-Yang Liao. NMR-spectroscopy-based metabonomic approach to the analysis of Bay41-4109, a novel anti-HBV compound, induced hepatotoxicity in rats. Toxicology letters. 2007 Sep; 173(3):161-7. doi: 10.1016/j.toxlet.2007.07.010. [PMID: 17826925]
  • Gregory Kaler, David M Truong, Akash Khandelwal, Megha Nagle, Satish A Eraly, Peter W Swaan, Sanjay K Nigam. Structural variation governs substrate specificity for organic anion transporter (OAT) homologs. Potential remote sensing by OAT family members. The Journal of biological chemistry. 2007 Aug; 282(33):23841-53. doi: 10.1074/jbc.m703467200. [PMID: 17553798]
  • Hye-Ran Yoon. Two step derivatization for the analyses of organic, amino acids and glycines on filter paper plasma by GC-MS/SIM. Archives of pharmacal research. 2007 Mar; 30(3):387-95. doi: 10.1007/bf02977623. [PMID: 17424948]
  • 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]
  • Anton Pohanka, Audrius Menkis, Jolanta Levenfors, Anders Broberg. Low-abundance kutznerides from Kutzneria sp. 744. Journal of natural products. 2006 Dec; 69(12):1776-81. doi: 10.1021/np0604331. [PMID: 17190458]
  • 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]
  • S H Korman, H Mandel, A Gutman. Characteristic urine organic acid profile in peroxisomal biogenesis disorders. Journal of inherited metabolic disease. 2000 Jun; 23(4):425-8. doi: 10.1023/a:1005624523611. [PMID: 10896310]
  • K R Kim, J Lee, D Ha, J Jeon, H G Park, J H Kim. Enantiomeric separation and discrimination of 2-hydroxy acids as O-trifluoroacetylated (S)-(+)-3-methyl-2-butyl esters by achiral dual-capillary column gas chromatography. Journal of chromatography. A. 2000 Mar; 874(1):91-100. doi: 10.1016/s0021-9673(00)00072-8. [PMID: 10768503]
  • M Wajner, J L Schlottfeldt, K Ckless, C M Wannmacher. Immunosuppressive effects of organic acids accumulating in patients with maple syrup urine disease. Journal of inherited metabolic disease. 1995; 18(2):165-8. doi: 10.1007/bf00711757. [PMID: 7564237]
  • T Nakanishi, A Shimizu. Identification of 3-hydroxyisovalerylcarnitine in the urine of a patient with multiple carboxylase deficiency. Annals of clinical biochemistry. 1993 May; 30 ( Pt 3)(?):318-20. doi: 10.1177/000456329303000315. [PMID: 8517616]
  • V Walker, G A Mills. Effects of birth asphyxia on urinary organic acid excretion. Biology of the neonate. 1992; 61(3):162-72. doi: 10.1159/000243739. [PMID: 1610944]
  • L Hagenfeldt, A S Naglo. New conjugated urinary metabolites in intermediate type maple syrup urine disease. Clinica chimica acta; international journal of clinical chemistry. 1987 Oct; 169(1):77-83. doi: 10.1016/0009-8981(87)90395-0. [PMID: 3677437]
  • 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]
  • T Kuhara, T Shinka, Y Inoue, M Matsumoto, M Yoshino, Y Sakaguchi, I Matsumoto. Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clinica chimica acta; international journal of clinical chemistry. 1983 Sep; 133(2):133-40. doi: 10.1016/0009-8981(83)90398-4. [PMID: 6688766]