| General Information of MET (ID: META00127) |
| Name |
(S)-3-Hydroxyisobutyric acid
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| Synonyms |
Click to Show/Hide Synonyms of This Metabolite
(2S)-3-Hydroxy-2-methylpropanoic acid; (2S)-3-Hydroxy-2-methylpropionic acid; (S)-3-Hydroxy-2-methylpropanoate; (S)-3-Hydroxy-2-methylpropanoic acid; (S)-3-Hydroxy-2-methylpropionate; (S)-3-Hydroxy-2-methylpropionic acid; (S)-3-Hydroxyisobutyrate; (S)-3-Hydroxyisobutyric acid; 2-(Hydroxymethyl)propanoic acid; 2-(Hydroxymethyl)propionic acid; 2-Methyl-3-hydroxypropanoic acid; 2-Methyl-3-hydroxypropionic acid; 2-Methyl-L-(+)-hydracrylate; 2-Methyl-L-(+)-hydracrylic acid; 3-HIBA; 3-Hydroxy(iso)butyric acid; 3-Hydroxy-2-isobutyrate; 3-Hydroxy-2-isobutyric acid; 3-Hydroxy-2-methyl-(S)-propanoate; 3-Hydroxy-2-methyl-(S)-propanoic acid; 3-Hydroxy-2-methylpropanoate; 3-Hydroxy-2-methylpropanoic acid; 3-Hydroxy-2-methylpropionic acid; 3-Hydroxy-isobutyrate; 3-Hydroxyisobutyrate; 3-Hydroxyisobutyric acid; DL-3-Hydroxyisobutyric acid
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| Source |
Endogenous;Escherichia Coli Metabolite;Yeast Metabolite;Food
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| Structure Type |
Beta hydroxy acids and derivatives (Click to Show/Hide the Complete Structure Type Hierarchy)
Organic acids and derivatives
Hydroxy acids and derivatives
Beta hydroxy acids and derivatives
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| PubChem CID |
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| HMDB ID |
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| Formula |
C4H8O3
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| Structure |
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3D MOL
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2D MOL
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Click to Show/Hide the Molecular/Functional Data (External Links/Property/Function) of This Metabolite
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| KEGG ID |
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| ChEBI ID |
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| FooDB ID |
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| ChemSpider ID |
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| METLIN ID |
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| Physicochemical Properties |
Molecular Weight |
104.1 |
Topological Polar Surface Area |
57.5 |
| XlogP |
-0.4 |
Complexity |
69.3 |
| Heavy Atom Count |
7 |
Rotatable Bond Count |
2 |
| Hydrogen Bond Donor Count |
2 |
Hydrogen Bond Acceptor Count |
3 |
| Function |
(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. 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. 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. 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.
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Regulatory Network
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Induced Change
