Details of Metabolite

General Information of MET (ID: META00249)
Name 2-Methylbutyroylcarnitine
Synonyms   Click to Show/Hide Synonyms of This Metabolite
(2-Methylbutyryl)carnitine; 3-[(2-Methylbutanoyl)oxy]-4-(trimethylammonio)butanoate; 3-[(2-Methylbutanoyl)oxy]-4-(trimethylammonio)butanoic acid; L-2-Methylbutyrate (3-carboxy-2-hydroxypropyl)trimethyl-hydroxide ammonium inner salt; Methylbutyroyl-carnitine
Source Endogenous;Fatty acyls;Food
Structure Type   Fatty acid esters  (Click to Show/Hide the Complete Structure Type Hierarchy)
Lipids and lipid-like molecules
Fatty Acyls
Fatty acid esters
PubChem CID
6426901
HMDB ID
HMDB0000378
Formula
C12H23NO4
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
73026
FooDB ID
FDB021996
ChemSpider ID
4932320
METLIN ID
5367
Physicochemical Properties Molecular Weight 245.32 Topological Polar Surface Area 66.4
XlogP 1.8 Complexity 265
Heavy Atom Count 17 Rotatable Bond Count 7
Hydrogen Bond Donor Count N.A. Hydrogen Bond Acceptor Count 4
Function
2-Methylbutyroylcarnitine is a member of the class of compounds known as acylcarnitines. Acylcarnitines are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond. Thus, 2-Methylbutyroylcarnitine is considered to be a fatty ester lipid molecule. 2-Methylbutyroylcarnitine is practically insoluble (in water) and a weakly acidic compound (based on its pKa). 2-Methylbutyroylcarnitine can be found in urine. Within the cell, 2-Methylbutyroylcarnitine is primarily located in the membrane (predicted from logP). It can also be found in the extracellular space. Usually not detected in normal individuals, the elevation of 2-methylbutyrylcarnitine suggests a deficiency of a dehydrogenase specific for isobutyryl-CoA, important in the differential diagnosis of branched chain organic acidurias by analysis of urinary organic acids and acylcarnitines in plasma or dried blood spots (The Metabolic and Molecular Bases of Inherited Disease, Chapter 93).
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-Methylbutyroylcarnitine concentration: decrease (FC = 0.67)
                      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-methylbutyroylcarnitine levels compared with control group.
      Hydrolases (EC 3)
            Sulfatase sulf-1 (SULF1) 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 Knockdown (shRNA) of SULF1
                      Induced Change 2-Methylbutyroylcarnitine concentration: decrease (FC = 0.34 / 0.38)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Ovarian cancer [ICD-11: 2C73]
                      Details It is reported that knockdown of SULF1 leads to the decrease of 2-methylbutyroylcarnitine 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 Erratum to: Loss of HSulf-1 promotes altered lipid metabolism in ovarian cancer. Cancer Metab. 2014 Nov 4;2:24.

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