Details of Metabolite

General Information of MET (ID: META00253)
Name 2-Hydroxy-3-methylbutyric acid
Synonyms   Click to Show/Hide Synonyms of This Metabolite
2-Hydroxy-3-methyl-butyric acid; 2-Hydroxy-3-methylbutanoate; 2-Hydroxy-3-methylbutanoic acid; 2-Hydroxy-3-methylbutyrate; 2-Hydroxyisopentanoate; 2-Hydroxyisopentanoic acid; 2-Hydroxyisovalerate; 2-Hydroxyisovaleric acid; 2-Hydroxyisovaleric acid, (R)-isomer; 2-Hydroxyisovaleric acid, (S)-isomer; 2-Hydroxyisovaleric acid, calcium (2:1) salt; 2-Hydroxyisovaleric acid, monosodium salt, (S)-isomer; 2-Oxyisovalerate; 2-Oxyisovaleric acid; 3-Methyl-2-hydroxybutyrate; 3-Methyl-2-hydroxybutyric acid; DL-2-Hydroxy-3-methylbutanoate; DL-2-Hydroxy-3-methylbutanoic acid; DL-2-Hydroxyisovalerate; DL-2-Hydroxyisovaleric acid; DL-a-Hydroxyisovalerate; DL-a-Hydroxyisovaleric acid; DL-alpha-Hydroxyisovalerate; DL-alpha-Hydroxyisovaleric acid; a-Hydroxyisovalerate; a-Hydroxyisovaleric acid; alpha-Hydroxyisovaleric acid
Source Endogenous;Yeast Metabolite;Fatty acyls;Food
Structure Type   Fatty acids and conjugates  (Click to Show/Hide the Complete Structure Type Hierarchy)
Lipids and lipid-like molecules
Fatty Acyls
Fatty acids and conjugates
PubChem CID
99823
HMDB ID
HMDB0000407
Formula
C5H10O3
Structure
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3D MOL 2D MOL
  Click to Show/Hide the Molecular/Functional Data (External Links/Property/Function) of This Metabolite
ChEBI ID
60645
FooDB ID
FDB022023
ChemSpider ID
90190
METLIN ID
5396
Physicochemical Properties Molecular Weight 118.13 Topological Polar Surface Area 57.5
XlogP 0.5 Complexity 87.7
Heavy Atom Count 8 Rotatable Bond Count 2
Hydrogen Bond Donor Count 2 Hydrogen Bond Acceptor Count 3
Function
2-Hydroxy-3-methylbutyric acid (also known as 2-hydroxyisovaleric acid) is a metabolite found in the urine of patients with phenylketonuria , 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. 2-Hydroxyisovaleric acid has also been identified in the urine of patients with lactic acidosis and ketoacidosis , and in the urine of severely asphyxiated babies. 2-Hydroxyisovaleric acid originates mainly from ketogenesis and from the metabolism of valine, leucine, and isoleucine. 2-Hydroxy-3-methylbutyric acid has been identified in the human placenta.
Regulatory Network
Full List of Protein(s) Regulating This Metabolite
      Apolipoprotein (Apo)
            Apolipoprotein A-II (APOA2) Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Protein   Info click to show the details of this protein
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Mutation (-265T >C(rs5082)) of APOA2
                      Induced Change 2-Hydroxy-3-methylbutyric acid concentration: decrease (FC = 0.54)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Obesity [ICD-11: 5B81]
                      Details It is reported that mutation (-265T >C(rs5082)) of APOA2 leads to the decrease of 2-hydroxy-3-methylbutyric acid levels compared with control group.
      Lyases (EC 4)
            Selenocysteine lyase (SCLY) Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Protein   Info click to show the details of this protein
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [2]
                      Introduced Variation Knockout of Scly
                      Induced Change 2-Hydroxy-3-methylbutyric acid concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Healthy individual
                      Details It is reported that knockout of Scly leads to the decrease of 2-hydroxy-3-methylbutyric acid levels compared with control group.
References
1 Epigenomics and metabolomics reveal the mechanism of the APOA2-saturated fat intake interaction affecting obesity. Am J Clin Nutr. 2018 Jul 1;108(1):188-200.
2 Combined Omics Reveals That Disruption of the Selenocysteine Lyase Gene Affects Amino Acid Pathways in Mice. Nutrients. 2019 Oct 26;11(11):2584.

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