Details of Metabolite

General Information of MET (ID: META00634)
Name 3-Hydroxybutyrylcarnitine
Synonyms   Click to Show/Hide Synonyms of This Metabolite
(3S)-3-{[(3R)-3-hydroxybutanoyl]oxy}-4-(trimethylazaniumyl)butanoic acid; (3S)-Hydroxybutyrylcarnitine; (S)-3-Hydroxybutyrylcarnitine; 3-Hydroxybutyrylcarnitine; Hydroxybutyrylcarnitine
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
HMDB ID
HMDB0013127
Formula
C11H21NO5
Structure
2D MOL
Function
3-Hydroxybutyrylcarnitine, also known as hydroxybutyrylcarnitine, is classified as a member of the acyl carnitines. Acyl carnitines are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond. 3-Hydroxybutyrylcarnitine is considered to be a slightly soluble (in water) and a weak acidic compound. 3-Hydroxybutyrylcarnitine is a fatty ester lipid molecule. 3-Hydroxybutyrylcarnitine can be found in blood. Within a cell, 3-hydroxybutyrylcarnitine is primarily located in the extracellular space and near the membrane.
Regulatory Network
Full List of Protein(s) Regulating This Metabolite
      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 [1]
                      Introduced Variation Mutation of ALDH1L2
                      Induced Change 3-Hydroxybutyrylcarnitine concentration: increase (FC = 2.86)
                      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 3-hydroxybutyrylcarnitine levels compared with control group.
References
1 Deleterious mutations in ALDH1L2 suggest a novel cause for neuro-ichthyotic syndrome. NPJ Genom Med. 2019 Jul 23;4:17.

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