Details of Metabolite

General Information of MET (ID: META00268)
Name 3-Methylglutarylcarnitine
Synonyms   Click to Show/Hide Synonyms of This Metabolite
(3R)-3-{[(3R)-4-carboxy-3-methylbutanoyl]oxy}-4-(trimethylazaniumyl)butanoic acid; 3-Methylglutarylcarnitine; Methylglutarylcarnitine; O-3-Methylglutarylcarnitine
Source Aliphatic acyclic compounds
Structure Type   Fatty acid esters  (Click to Show/Hide the Complete Structure Type Hierarchy)
Lipids and lipid-like molecules
Fatty Acyls
Fatty acid esters
HMDB ID
HMDB0000552
Formula
C13H23NO6
Structure
2D MOL
FooDB ID
FDB022112
Function
3-Methylglutarylcarnitine belongs to the class of organic compounds known as acylcarnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond 3-Methylglutarylcarnitine is an extremely weak basic (essentially neutral) compound (based on its pKa). 3-Methylglutarylcarnitine is a diagnostic metabolite of 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency. It is also identified in the urine of patients with Reye-like syndrome.
Regulatory Network
Full List of Protein(s) Regulating This Metabolite
      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 [1]
                      Introduced Variation Knockdown (shRNA) of SULF1
                      Induced Change 3-Methylglutarylcarnitine concentration: decrease (FC = 0.49 / 0.58)
                      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 3-methylglutarylcarnitine levels compared with control group.
References
1 Erratum to: Loss of HSulf-1 promotes altered lipid metabolism in ovarian cancer. Cancer Metab. 2014 Nov 4;2:24.

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