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Showing metabocard for 4-Trimethylammoniobutanoic acid (HMDB0001161)

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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2023-02-21 17:15:29 UTC
HMDB IDHMDB0001161
Secondary Accession Numbers
  • HMDB0006831
  • HMDB01161
  • HMDB06831
Metabolite Identification
Common Name4-Trimethylammoniobutanoic acid
Description4-Trimethylammoniobutanoic acid, also known as gamma-butyrobetaine (GBB) or 3-dehydroxycarnitine, is a highly water-soluble derivative of gamma-aminobutyric acid (GABA). It is also a precursor of L-carnitine. It is a substrate of gamma butyrobetaine hydroxylase/dioxygenase (also known as BBOX) which catalyzes the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthesis pathway. Carnitine is essential for the transport of activated fatty acids across the mitochondrial membrane during mitochondrial beta-oxidation. Numerous disorders have been described that lead to disturbances in energy production and in intermediary metabolism which are characterized by the production and excretion of unusual acylcarnitines. A mutation in the gene coding for carnitine-acylcarnitine translocase, or the OCTN2 transporter aetiologically, causes a carnitine deficiency that results in poor intestinal absorption of dietary L-carnitine, impaired reabsorption by the kidney, and increased urinary loss. Determination of the qualitative pattern of acylcarnitines can be of diagnostic and therapeutic importance. The betaine structure of carnitine requires special analytical procedures for recording. The ionic nature of L-carnitine causes a high water solubility which decreases with increasing chain length of the ester group in the acylcarnitines. Therefore, the distribution of L-carnitine and acylcarnitines in various organs is defined by their function and their physicochemical properties as well. High-performance liquid chromatography (HPLC) permits screening for free and total carnitine, as well as complete quantitative acylcarnitine determination, including the long-chain acylcarnitine profile (PMID: 17508264 , Monatshefte fuer Chemie (2005), 136(8), 1279-1291., Int J Mass Spectrom. 1999;188:39-52.).
Structure
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MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0001161 (4-Trimethylammoniobutanoic acid)

  Mrv1652307191923392D          

 10  9  0  0  0  0            999 V2000
    9.7697   -6.1666    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   10.4819   -5.7504    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   12.6273   -6.1581    0.0000 N   0  3  0  0  0  0  0  0  0  0  0  0
    9.0574   -5.7547    0.0000 O   0  5  0  0  0  0  0  0  0  0  0  0
    9.7697   -6.9905    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   11.1985   -6.1623    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   11.9108   -5.7461    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   13.0350   -5.4372    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   13.3482   -6.5657    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   12.2197   -6.8746    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  2  1  1  0  0  0  0
  4  1  1  0  0  0  0
  5  1  2  0  0  0  0
  6  2  1  0  0  0  0
  7  3  1  0  0  0  0
  8  3  1  0  0  0  0
  9  3  1  0  0  0  0
 10  3  1  0  0  0  0
  7  6  1  0  0  0  0
M  CHG  2   3   1   4  -1
M  END
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3D MOL for HMDB0001161 (4-Trimethylammoniobutanoic acid)

HMDB0001161
     RDKit          3D
4-Trimethylammoniobutanoic acid
 25 24  0  0  0  0  0  0  0  0999 V2000
   -2.1743   -0.6734    0.7003 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9450   -0.1669    0.0502 N   0  0  0  0  0  4  0  0  0  0  0  0
   -0.9050    1.2566    0.4394 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1237   -0.2838   -1.3560 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.1937   -0.7963    0.6249 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.3737   -1.0407   -0.2310 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.9365    0.2183   -0.8011 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.4064    1.2040    0.1913 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.3840    1.0055    1.4216 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.8990    2.4150   -0.2680 O   0  0  0  0  0  1  0  0  0  0  0  0
   -2.0097   -0.5736    1.7887 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3192   -1.7385    0.4900 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0163   -0.0603    0.3716 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3404    1.3489    1.3981 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4040    1.8660   -0.3091 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9308    1.6130    0.6640 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1164    0.7059   -1.8808 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4639   -0.9788   -1.8786 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1731   -0.6604   -1.5575 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1818   -1.8212    0.9785 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.4633   -0.2193    1.5601 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.1672   -1.7502   -1.0566 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.2214   -1.4883    0.3923 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.8297   -0.0503   -1.4293 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.2289    0.6686   -1.5329 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  2  5  1  0
  5  6  1  0
  6  7  1  0
  7  8  1  0
  8  9  2  0
  8 10  1  0
  1 11  1  0
  1 12  1  0
  1 13  1  0
  3 14  1  0
  3 15  1  0
  3 16  1  0
  4 17  1  0
  4 18  1  0
  4 19  1  0
  5 20  1  0
  5 21  1  0
  6 22  1  0
  6 23  1  0
  7 24  1  0
  7 25  1  0
M  CHG  2   2   1  10  -1
M  END
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3D SDF for HMDB0001161 (4-Trimethylammoniobutanoic acid)

  Mrv1652307191923392D          

 10  9  0  0  0  0            999 V2000
    9.7697   -6.1666    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   10.4819   -5.7504    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   12.6273   -6.1581    0.0000 N   0  3  0  0  0  0  0  0  0  0  0  0
    9.0574   -5.7547    0.0000 O   0  5  0  0  0  0  0  0  0  0  0  0
    9.7697   -6.9905    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   11.1985   -6.1623    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   11.9108   -5.7461    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   13.0350   -5.4372    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   13.3482   -6.5657    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   12.2197   -6.8746    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  2  1  1  0  0  0  0
  4  1  1  0  0  0  0
  5  1  2  0  0  0  0
  6  2  1  0  0  0  0
  7  3  1  0  0  0  0
  8  3  1  0  0  0  0
  9  3  1  0  0  0  0
 10  3  1  0  0  0  0
  7  6  1  0  0  0  0
M  CHG  2   3   1   4  -1
M  END
> <DATABASE_ID>
HMDB0001161

> <DATABASE_NAME>
hmdb

> <SMILES>
C[N+](C)(C)CCCC([O-])=O

> <INCHI_IDENTIFIER>
InChI=1S/C7H15NO2/c1-8(2,3)6-4-5-7(9)10/h4-6H2,1-3H3

> <INCHI_KEY>
JHPNVNIEXXLNTR-UHFFFAOYSA-N

> <FORMULA>
C7H15NO2

> <MOLECULAR_WEIGHT>
145.1995

> <EXACT_MASS>
145.110278729

> <JCHEM_ACCEPTOR_COUNT>
2

> <JCHEM_ATOM_COUNT>
25

> <JCHEM_AVERAGE_POLARIZABILITY>
16.19807438182574

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
0

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
4-(trimethylazaniumyl)butanoate

> <ALOGPS_LOGP>
-3.15

> <JCHEM_LOGP>
-3.968509901138413

> <ALOGPS_LOGS>
-2.95

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
0

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.457564906027561

> <JCHEM_POLAR_SURFACE_AREA>
40.129999999999995

> <JCHEM_REFRACTIVITY>
62.277800000000006

> <JCHEM_ROTATABLE_BOND_COUNT>
4

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
2.25e-01 g/l

> <JCHEM_TRADITIONAL_IUPAC>
butyrobetaine

> <JCHEM_VEBER_RULE>
0

$$$$
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3D-SDF for HMDB0001161 (4-Trimethylammoniobutanoic acid)

HMDB0001161
     RDKit          3D
4-Trimethylammoniobutanoic acid
 25 24  0  0  0  0  0  0  0  0999 V2000
   -2.1743   -0.6734    0.7003 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9450   -0.1669    0.0502 N   0  0  0  0  0  4  0  0  0  0  0  0
   -0.9050    1.2566    0.4394 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1237   -0.2838   -1.3560 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.1937   -0.7963    0.6249 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.3737   -1.0407   -0.2310 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.9365    0.2183   -0.8011 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.4064    1.2040    0.1913 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.3840    1.0055    1.4216 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.8990    2.4150   -0.2680 O   0  0  0  0  0  1  0  0  0  0  0  0
   -2.0097   -0.5736    1.7887 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3192   -1.7385    0.4900 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0163   -0.0603    0.3716 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3404    1.3489    1.3981 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4040    1.8660   -0.3091 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9308    1.6130    0.6640 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1164    0.7059   -1.8808 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4639   -0.9788   -1.8786 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1731   -0.6604   -1.5575 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1818   -1.8212    0.9785 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.4633   -0.2193    1.5601 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.1672   -1.7502   -1.0566 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.2214   -1.4883    0.3923 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.8297   -0.0503   -1.4293 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.2289    0.6686   -1.5329 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  2  5  1  0
  5  6  1  0
  6  7  1  0
  7  8  1  0
  8  9  2  0
  8 10  1  0
  1 11  1  0
  1 12  1  0
  1 13  1  0
  3 14  1  0
  3 15  1  0
  3 16  1  0
  4 17  1  0
  4 18  1  0
  4 19  1  0
  5 20  1  0
  5 21  1  0
  6 22  1  0
  6 23  1  0
  7 24  1  0
  7 25  1  0
M  CHG  2   2   1  10  -1
M  END
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PDB for HMDB0001161 (4-Trimethylammoniobutanoic acid)

HEADER    PROTEIN                                 19-JUL-19   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   19-JUL-19         0                                  
HETATM    1  C   UNK     0      18.237 -11.511   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0      19.566 -10.734   0.000  0.00  0.00           C+0
HETATM    3  N   UNK     0      23.571 -11.495   0.000  0.00  0.00           N+1
HETATM    4  O   UNK     0      16.907 -10.742   0.000  0.00  0.00           O-1
HETATM    5  O   UNK     0      18.237 -13.049   0.000  0.00  0.00           O+0
HETATM    6  C   UNK     0      20.904 -11.503   0.000  0.00  0.00           C+0
HETATM    7  C   UNK     0      22.233 -10.726   0.000  0.00  0.00           C+0
HETATM    8  C   UNK     0      24.332 -10.149   0.000  0.00  0.00           C+0
HETATM    9  C   UNK     0      24.917 -12.256   0.000  0.00  0.00           C+0
HETATM   10  C   UNK     0      22.810 -12.833   0.000  0.00  0.00           C+0
CONECT    1    2    4    5                                                  
CONECT    2    1    6                                                       
CONECT    3    7    8    9   10                                             
CONECT    4    1                                                            
CONECT    5    1                                                            
CONECT    6    2    7                                                       
CONECT    7    3    6                                                       
CONECT    8    3                                                            
CONECT    9    3                                                            
CONECT   10    3                                                            
MASTER        0    0    0    0    0    0    0    0   10    0   18    0
END
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3D PDB for HMDB0001161 (4-Trimethylammoniobutanoic acid)

COMPND    HMDB0001161
HETATM    1  C1  UNL     1      -2.174  -0.673   0.700  1.00  0.00           C  
HETATM    2  N1  UNL     1      -0.945  -0.167   0.050  1.00  0.00           N1+
HETATM    3  C2  UNL     1      -0.905   1.257   0.439  1.00  0.00           C  
HETATM    4  C3  UNL     1      -1.124  -0.284  -1.356  1.00  0.00           C  
HETATM    5  C4  UNL     1       0.194  -0.796   0.625  1.00  0.00           C  
HETATM    6  C5  UNL     1       1.374  -1.041  -0.231  1.00  0.00           C  
HETATM    7  C6  UNL     1       1.937   0.218  -0.801  1.00  0.00           C  
HETATM    8  C7  UNL     1       2.406   1.204   0.191  1.00  0.00           C  
HETATM    9  O1  UNL     1       2.384   1.006   1.422  1.00  0.00           O  
HETATM   10  O2  UNL     1       2.899   2.415  -0.268  1.00  0.00           O1-
HETATM   11  H1  UNL     1      -2.010  -0.574   1.789  1.00  0.00           H  
HETATM   12  H2  UNL     1      -2.319  -1.738   0.490  1.00  0.00           H  
HETATM   13  H3  UNL     1      -3.016  -0.060   0.372  1.00  0.00           H  
HETATM   14  H4  UNL     1      -0.340   1.349   1.398  1.00  0.00           H  
HETATM   15  H5  UNL     1      -0.404   1.866  -0.309  1.00  0.00           H  
HETATM   16  H6  UNL     1      -1.931   1.613   0.664  1.00  0.00           H  
HETATM   17  H7  UNL     1      -1.116   0.706  -1.881  1.00  0.00           H  
HETATM   18  H8  UNL     1      -0.464  -0.979  -1.879  1.00  0.00           H  
HETATM   19  H9  UNL     1      -2.173  -0.660  -1.558  1.00  0.00           H  
HETATM   20  H10 UNL     1      -0.182  -1.821   0.978  1.00  0.00           H  
HETATM   21  H11 UNL     1       0.463  -0.219   1.560  1.00  0.00           H  
HETATM   22  H12 UNL     1       1.167  -1.750  -1.057  1.00  0.00           H  
HETATM   23  H13 UNL     1       2.221  -1.488   0.392  1.00  0.00           H  
HETATM   24  H14 UNL     1       2.830  -0.050  -1.429  1.00  0.00           H  
HETATM   25  H15 UNL     1       1.229   0.669  -1.533  1.00  0.00           H  
CONECT    1    2   11   12   13
CONECT    2    3    4    5
CONECT    3   14   15   16
CONECT    4   17   18   19
CONECT    5    6   20   21
CONECT    6    7   22   23
CONECT    7    8   24   25
CONECT    8    9    9   10
END
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SMILES for HMDB0001161 (4-Trimethylammoniobutanoic acid)

C[N+](C)(C)CCCC([O-])=O
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INCHI for HMDB0001161 (4-Trimethylammoniobutanoic acid)

InChI=1S/C7H15NO2/c1-8(2,3)6-4-5-7(9)10/h4-6H2,1-3H3
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View in JSmolView NMR AssignmentsView Stereo Labels
Synonyms
Chemical FormulaC7H15NO2
Average Molecular Weight145.1995
Monoisotopic Molecular Weight145.110278729
IUPAC Name4-(trimethylazaniumyl)butanoate
Traditional Namebutyrobetaine
CAS Registry Number407-64-7
SMILES
C[N+](C)(C)CCCC([O-])=O
InChI Identifier
InChI=1S/C7H15NO2/c1-8(2,3)6-4-5-7(9)10/h4-6H2,1-3H3
InChI KeyJHPNVNIEXXLNTR-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as straight chain fatty acids. These are fatty acids with a straight aliphatic chain.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
Direct ParentStraight chain fatty acids
Alternative Parents
Substituents
  • Straight chain fatty acid
  • Quaternary ammonium salt
  • Tetraalkylammonium salt
  • Carboxylic acid salt
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxide
  • Amine
  • Hydrocarbon derivative
  • Organic salt
  • Organic nitrogen compound
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Organopnictogen compound
  • Organic oxygen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effectNot Available
Disposition
Biological locationSource
Process
Naturally occurring process
RoleNot Available
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic Properties

Experimental Collision Cross Sections

Adduct TypeData SourceCCS Value (Å2)Reference
[M+H]+Not Available130.397http://allccs.zhulab.cn/database/detail?ID=AllCCS00001979
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Kidney
  • Liver
  • Placenta
Pathways
Normal Concentrations
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Colorectal cancer		 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
Associated Disorders and Diseases
Disease References
Colorectal cancer
  1. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
  2. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276 ]
  3. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050 ]
Associated OMIM IDs
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB024107
KNApSAcK IDNot Available
Chemspider ID705
KEGG Compound IDC01181
BioCyc IDGAMMA-BUTYROBETAINE
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound725
PDB IDNot Available
ChEBI ID16244
Food Biomarker OntologyNot Available
VMH ID4TMEABUTN
MarkerDB IDNot Available
Good Scents IDNot Available
References
Synthesis ReferenceKawamura, Masao; Akutsu, Seiichi; Fukuda, Hirosuke; Hata, Hiroyuki; Morishita, Tsuyoshi; Kano, Kenji; Nishimori, Hirokuni. Manufacture of g-butyrobetaine by fermentation. Jpn. Kokai Tokkyo Koho (1987), 6 pp.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Hewawasam P, Ding M, Chen N, King D, Knipe J, Pajor L, Ortiz A, Gribkoff VK, Starrett J: Synthesis of water-soluble prodrugs of BMS-191011: a maxi-K channel opener targeted for post-stroke neuroprotection. Bioorg Med Chem Lett. 2003 May 19;13(10):1695-8. [PubMed:12729644 ]
  2. Siliprandi N, Ciman M, Sartorelli L: Myocardial carnitine transport. Basic Res Cardiol. 1987;82 Suppl 1:53-62. [PubMed:3311009 ]
  3. Rebouche CJ, Engel AG: Significance of renal gamma-butyrobetaine hydroxylase for carnitine biosynthesis in man. J Biol Chem. 1980 Sep 25;255(18):8700-5. [PubMed:6773946 ]
  4. Terada N, Inoue F, Okochi M, Nakajima H, Kizaki Z, Kinugasa A, Sawada T: Measurement of carnitine precursors, epsilon-trimethyllysine and gamma-butyrobetaine in human serum by tandem mass spectrometry. J Chromatogr B Biomed Sci Appl. 1999 Aug 6;731(1):89-95. [PubMed:10491993 ]
  5. Rebouche CJ, Chenard CA: Metabolic fate of dietary carnitine in human adults: identification and quantification of urinary and fecal metabolites. J Nutr. 1991 Apr;121(4):539-46. [PubMed:2007906 ]
  6. Pierre G, Macdonald A, Gray G, Hendriksz C, Preece MA, Chakrapani A: Prospective treatment in carnitine-acylcarnitine translocase deficiency. J Inherit Metab Dis. 2007 Oct;30(5):815. Epub 2007 May 12. [PubMed:17508264 ]
  7. Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]

Enzymes

Enzyme Details
1. 4-trimethylaminobutyraldehyde dehydrogenase
General function:
Involved in oxidoreductase activity
Specific function:
Converts gamma-trimethylaminobutyraldehyde into gamma-butyrobetaine. Catalyzes the irreversible oxidation of a broad range of aldehydes to the corresponding acids in an NAD-dependent reaction.
Gene Name:
ALDH9A1
Uniprot ID:
P49189
Molecular weight:
56291.485
Reactions
4-Trimethylammoniobutanal + NAD + Water → 4-Trimethylammoniobutanoic acid + NADHdetails
4-Trimethylammoniobutanal + NAD + Water → 4-Trimethylammoniobutanoic acid + NADH + Hydrogen Iondetails
Enzyme Details
2. Alpha-aminoadipic semialdehyde dehydrogenase
General function:
Involved in oxidoreductase activity
Specific function:
Multifunctional enzyme mediating important protective effects. Metabolizes betaine aldehyde to betaine, an important cellular osmolyte and methyl donor. Protects cells from oxidative stress by metabolizing a number of lipid peroxidation-derived aldehydes. Involved in lysine catabolism.
Gene Name:
ALDH7A1
Uniprot ID:
P49419
Molecular weight:
58486.74
Reactions
4-Trimethylammoniobutanal + NAD + Water → 4-Trimethylammoniobutanoic acid + NADH + Hydrogen Iondetails
Enzyme Details
3. Aldehyde dehydrogenase family 1 member A3
General function:
Involved in oxidoreductase activity
Specific function:
Recognizes as substrates free retinal and cellular retinol-binding protein-bound retinal. Seems to be the key enzyme in the formation of an RA gradient along the dorso-ventral axis during the early eye development and also in the development of the olfactory system (By similarity).
Gene Name:
ALDH1A3
Uniprot ID:
P47895
Molecular weight:
56107.995
Enzyme Details
4. Aldehyde dehydrogenase, mitochondrial
General function:
Involved in oxidoreductase activity
Specific function:
Not Available
Gene Name:
ALDH2
Uniprot ID:
P05091
Molecular weight:
56380.93
Reactions
4-Trimethylammoniobutanal + NAD + Water → 4-Trimethylammoniobutanoic acid + NADH + Hydrogen Iondetails
Enzyme Details
5. Fatty aldehyde dehydrogenase
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the oxidation of long-chain aliphatic aldehydes to fatty acids. Active on a variety of saturated and unsaturated aliphatic aldehydes between 6 and 24 carbons in length. Responsible for conversion of the sphingosine 1-phosphate (S1P) degradation product hexadecenal to hexadecenoic acid.
Gene Name:
ALDH3A2
Uniprot ID:
P51648
Molecular weight:
54847.36
Reactions
4-Trimethylammoniobutanal + NAD + Water → 4-Trimethylammoniobutanoic acid + NADH + Hydrogen Iondetails
Enzyme Details
6. Aldehyde dehydrogenase X, mitochondrial
General function:
Involved in oxidoreductase activity
Specific function:
ALDHs play a major role in the detoxification of alcohol-derived acetaldehyde. They are involved in the metabolism of corticosteroids, biogenic amines, neurotransmitters, and lipid peroxidation.
Gene Name:
ALDH1B1
Uniprot ID:
P30837
Molecular weight:
57248.96
Reactions
4-Trimethylammoniobutanal + NAD + Water → 4-Trimethylammoniobutanoic acid + NADH + Hydrogen Iondetails
Enzyme Details
7. Gamma-butyrobetaine dioxygenase
General function:
Involved in iron ion binding
Specific function:
Catalyzes the formation of L-carnitine from gamma-butyrobetaine.
Gene Name:
BBOX1
Uniprot ID:
O75936
Molecular weight:
44714.6
Reactions
4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen → L-Carnitine + Succinic acid + CO(2)details
4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen → L-Carnitine + Succinic acid + Carbon dioxidedetails