Details of Metabolite

General Information of MET (ID: META00465)
Name Butyrylcarnitine
Synonyms   Click to Show/Hide Synonyms of This Metabolite
(3R)-3-(Butyryloxy)-4-(trimethylammonio)butanoate; (3R)-3-(Butyryloxy)-4-(trimethylammonio)butanoic acid; Butylcarnitine; Butyryl-L-carnitine; Butyrylcarnitine; L-Carnitine butyryl ester; N-Butyryl-L-(-)-carnitin; O-Butanoyl-(R)-carnitine; O-Butanoyl-carnitine; O-Butanoylcarnitine
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
PubChem CID
213144
HMDB ID
HMDB0002013
Formula
C11H21NO4
Structure
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3D MOL 2D MOL
  Click to Show/Hide the Molecular/Functional Data (External Links/Property/Function) of This Metabolite
KEGG ID
C02862
ChEBI ID
21949
FooDB ID
FDB022795
ChemSpider ID
184820
METLIN ID
6436
Physicochemical Properties Molecular Weight 231.29 Topological Polar Surface Area 66.4
XlogP 1.2 Complexity 239
Heavy Atom Count 16 Rotatable Bond Count 7
Hydrogen Bond Donor Count N.A. Hydrogen Bond Acceptor Count 4
Function
Butyrylcarnitine, also known as (3R)-3-(butyryloxy)-4-(trimethylammonio)butanoate or L-carnitine butyryl ester, is classified as a member of the acylcarnitines. Acylcarnitines are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond. Butyrylcarnitine is considered to be practically insoluble (in water) and acidic. Butyrylcarnitine is elevated in patients with short-chain acyl-CoA dehydrogenase (SCAD) deficiency, in infants with acute acidosis and generalized muscle weakness, and in middle-aged patients with chronic myopathy localized in muscle (OMIM: 201470).
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 Butyrylcarnitine concentration: increase (FC = 1.42)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Ovarian cancer [ICD-11: 2C73]
                      Details It is reported that knockdown of SULF1 leads to the increase of butyrylcarnitine levels compared with control group.
      Oxidoreductases (EC 1)
            Formyltetrahydrofolate dehydrogenase (ALDH1L2) 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 Mutation of ALDH1L2
                      Induced Change Butyrylcarnitine concentration: increase (FC = 3.85)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Genetic disorders of keratinisation [ICD-11: EC20]
                      Details It is reported that mutation (patients) of ALDH1L2 leads to the increase of butyrylcarnitine levels compared with control group.
            Glutamate-cysteine ligase modifier (GCLM) 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 [3]
                      Introduced Variation Knockout of Gclm
                      Induced Change Butyrylcarnitine concentration: decrease (FC = 0.52)
                      Summary Introduced Variation         Induced Change 
                      Disease Status Metabolic liver disease [ICD-11: 5C90]
                      Details It is reported that knockout of Gclm leads to the decrease of butyrylcarnitine 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.
2 Deleterious mutations in ALDH1L2 suggest a novel cause for neuro-ichthyotic syndrome. NPJ Genom Med. 2019 Jul 23;4:17.
3 Hepatic metabolic adaptation in a murine model of glutathione deficiency. Chem Biol Interact. 2019 Apr 25;303:1-6.

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