Details of Metabolite

General Information of MET (ID: META00245)
Name 2-Hydroxy-3-methylpentanoic acid
Synonyms   Click to Show/Hide Synonyms of This Metabolite
(2R,3R)-2-Hydroxy-3-methyl-pentanoate; (2R,3R)-2-Hydroxy-3-methyl-pentanoic acid; (2R,3R)-2-Hydroxy-3-methylpentanoate; (2R,3R)-2-Hydroxy-3-methylpentanoic acid; 2-Hydroxy-3-methyl-pentanoate; 2-Hydroxy-3-methyl-pentanoic acid; 2-Hydroxy-3-methyl-valerate; 2-Hydroxy-3-methyl-valeric acid; 2-Hydroxy-3-methylpentanoate; 2-Hydroxy-3-methylvalerate; 2-Hydroxy-3-methylvaleric acid; HMVA; a-Hydroxy-b-methylvalerate; a-Hydroxy-b-methylvaleric acid; alpha-Hydroxy-beta-methylvalerate; alpha-Hydroxy-beta-methylvaleric acid
Source Endogenous;Escherichia Coli Metabolite;Yeast Metabolite;Fatty acyls;Food;Microbial
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
10796774
HMDB ID
HMDB0000317
Formula
C6H12O3
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
89228
FooDB ID
FDB021944
ChemSpider ID
8972081
METLIN ID
5306
Physicochemical Properties Molecular Weight 132.16 Topological Polar Surface Area 57.5
XlogP 0.9 Complexity 101
Heavy Atom Count 9 Rotatable Bond Count 3
Hydrogen Bond Donor Count 2 Hydrogen Bond Acceptor Count 3
Function
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. 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). Maple syrup urine disease (MSUD) is an inherited metabolic disease predominantly characterized by neurological dysfunction including psychomotor/delay/mental retardation.
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-methylpentanoic acid concentration: decrease (FC = 0.55)
                      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-methylpentanoic 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.

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