3-Hydroxyisobutyric acid (BioDeep_00000014319)

Main id: BioDeep_00000004608

Secondary id: BioDeep_00000405187

human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite natural product


代谢物信息卡片


3-Hydroxy-2-methyl-(S)-propanoic acid

化学式: C4H8O3 (104.0473)
中文名称: 3-羟基异丁酸, (S)-3-羟基-2-甲基丙酸
谱图信息: 最多检出来源 Homo sapiens(blood) 22.51%

Reviewed

Last reviewed on 2024-07-19.

Cite this Page

3-Hydroxyisobutyric acid. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/(s)-3-hydroxyisobutyric_acid (retrieved 2024-12-22) (BioDeep RN: BioDeep_00000014319). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: CC(CO)C(=O)O
InChI: InChI=1S/C4H8O3/c1-3(2-5)4(6)7/h3,5H,2H2,1H3,(H,6,7)/t3-/m0/s1

描述信息

(S)-3-Hydroxyisobutyric acid (3-HIBA) (CAS: 2068-83-9) is an organic acid. 3-HIBA is an intermediate in L-valine metabolism. 3-HIBA plays an important role in the diagnosis of the very rare inherited metabolic diseases 3-hydroxyisobutyric aciduria (OMIM: 236795) and methylmalonic semialdehyde dehydrogenase deficiency (OMIM: 603178). Patients with 3-hydroxyisobutyric aciduria excrete a significant amount of 3-HIBA not only during the acute stage but also when stable. 3-Hydroxyisobutyric aciduria is caused by a 3-hydroxyisobutyryl-CoA dehydrogenase deficiency (PMID: 18329219). The severity of this disease varies from case to case. Most patients exhibit dysmorphic features, such as a small triangular face, a long philtrum, low set ears, and micrognathia (PMID: 10686279). Lactic acidemia is also found in the affected patients, indicating that mitochondrial dysfunction is involved. 3-HIBA appears to specifically inhibit the function of the respiratory chain complex I-III and mitochondrial creatine kinase (PMID: 18329219). BioTransformer predicts that 3-HIBA is a product of 2-methylpropanoic acid metabolism via a hydroxylation-of-terminal-methyl reaction catalyzed by CYP2B6 and CYP2E1 enzymes (PMID: 30612223).
(S)-3-Hydroxyisobutyric (3-HIBA) acid is an organic acid. 3-HIBA is an intermediate in the metabolic pathways of L-valine and L-thymine amino acids. 3-HIBA plays an important role in the diagnosis of the very rare inherited metabolic diseases 3-hydroxyisobutyric aciduria (OMIM 236795) and methylmalonic semialdehyde dehydrogenase deficiency (OMIM 603178). Patients with 3-hydroxyisobutyric aciduria excrete a significant amount of 3-HIBA not only during the acute stage but also when stable. The deficient enzyme in 3HiB-uria remains unclear. The severity of this disease varies from case to case. Most patients exhibit dysmorphic features, such as a small triangular face, a long philtrum, low set ears and micrognathia (PMID: 113770040, 10686279) [HMDB]
3-Hydroxyisobutyric acid is an important interorgan metabolite, an intermediate in the pathways of l-valine and thymine and a good gluconeogenic substrate.

同义名列表

37 个代谢物同义名

3-Hydroxy-2-methyl-(S)-propanoic acid; (2S)-3-Hydroxy-2-methylpropionic acid; (2S)-3-Hydroxy-2-methylpropanoic acid; (S)-3-Hydroxy-2-methylpropanoic acid; (±)-3-hydroxy-2-methylpropanoic acid; (±)-3-hydroxy-2-methylpropionic acid; (S)-3-Hydroxy-2-methylpropionic acid; L-(+)-beta-Hydroxyisobutyric acid; 3-Hydroxy-2-methyl-(S)-propanoate; 2-Methyl-3-hydroxypropanoic acid; (S)-3-Hydroxy-2-methylpropionate; 2-Methyl-3-hydroxypropionic acid; 3-Hydroxy-2-methylpropionic acid; (S)-3-Hydroxy-2-methylpropanoate; 3-Hydroxy-2-methylpropanoic acid; (S)-beta-Hydroxyisobutyric acid; 2-(Hydroxymethyl)propanoic acid; 2-(Hydroxymethyl)propionic acid; 2-Methyl-L-(+)-hydracrylic acid; L-(+)-Β-hydroxyisobutyric acid; (S)-3-Hydroxyisobutyric acid; (S)-Β-hydroxyisobutyric acid; 3-Hydroxy-2-methylpropanoate; 3-Hydroxy-2-isobutyric acid; 2-Methyl-L-(+)-hydracrylate; beta-Hydroxyisobutyric acid; DL-3-Hydroxyisobutyric acid; 3-Hydroxy(iso)butyric acid; Β-hydroxyisobutyric acid; 3-Hydroxyisobutyric acid; (S)-3-CHLOROSTYRENEOXIDE; (S)-3-Hydroxyisobutyrate; 3-Hydroxy-2-isobutyrate; 3-Hydroxy-isobutyrate; 3-Hydroxyisobutyrate; 3-HIBA; 3-Hydroxy-2-methylpropanoate



数据库引用编号

18 个数据库交叉引用编号

分类词条

相关代谢途径

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代谢反应

55 个相关的代谢反应过程信息。

Reactome(0)

BioCyc(0)

WikiPathways(0)

Plant Reactome(0)

INOH(2)

PlantCyc(0)

COVID-19 Disease Map(0)

PathBank(53)

PharmGKB(0)

15 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。

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