Details of Metabolite

General Information of MET (ID: META00328)
Name Propionylcarnitine
Synonyms   Click to Show/Hide Synonyms of This Metabolite
(-)-Propionylcarnitine; (3R)-3-(Propionyloxy)-4-(trimethylammonio)butanoate; (3R)-3-(Propionyloxy)-4-(trimethylammonio)butanoic acid; (R)-3-Carboxy-N,N,N-trimethyl-2-(1-oxopropoxy)-1-propanaminium inner salt; C3-Carnitine; L-Carnitine propionyl ester; L-Propionylcarnitine; Levocarnitine propil; O-Propanoyl-(R)-carnitine; O-Propanoyl-L-carnitine; O-Propanoyl-carnitine; O-Propanoylcarnitine; O-Propinoyl-L-carnitine; O-Propionyl-L-carnitine; Propanoyl-L-carnitine; Propionyl-L-carnitine; Propionylcarnitine
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
188824
HMDB ID
HMDB0000824
Formula
C10H19NO4
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
C03017
ChEBI ID
53210
FooDB ID
FDB022268
ChemSpider ID
164090
Physicochemical Properties Molecular Weight 217.26 Topological Polar Surface Area 66.4
XlogP 0.9 Complexity 227
Heavy Atom Count 15 Rotatable Bond Count 6
Hydrogen Bond Donor Count N.A. Hydrogen Bond Acceptor Count 4
Function
Propionylcarnitine (CAS: 17298-37-2), also known as C3-carnitine or O-propanoylcarnitine, 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. Thus, propionylcarnitine is considered to be a fatty ester lipid molecule. Specifically, propionylcarnitine is an optically active O-acylcarnitine compound having propanoyl as the acyl substituent and the (R)-configuration at the 3-position. Propionylcarnitine is a very hydrophobic molecule, practically insoluble (in water), and relatively neutral. Propionylcarnitine (along with methylmalonic acid) is present in high abundance in the urine of patients with methylmalonyl-CoA mutase (MUT) deficiency. MUT is a mitochondrial enzyme that catalyzes the isomerization of methylmalonyl-CoA into succinyl-CoA (OMIM: 609058). Propionylcarnitine is also found to be associated with propionic acidemia, which is an inborn error of metabolism.
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 Propionylcarnitine concentration: decrease (FC = 0.81)
                      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 propionylcarnitine 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 Propionylcarnitine concentration: increase (FC = 4)
                      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 propionylcarnitine 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 Deleterious mutations in ALDH1L2 suggest a novel cause for neuro-ichthyotic syndrome. NPJ Genom Med. 2019 Jul 23;4:17.

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