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Record Information
Version4.0
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-10-23 19:03:48 UTC
HMDB IDHMDB0001161
Secondary Accession Numbers
  • HMDB01161
Metabolite Identification
Common Name4-Trimethylammoniobutanoic acid
Description4-Trimethylammoniobutanoic acid or Gamma-butyrobetaine (GBB) is a highly water-soluble derivative of gamma-amino butyric acid. 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 biosynthetic pathway. Carnitine is essential for the transport of activated fatty acids across the mitochondrial membrane during mitochondrial beta-oxidation. Gamma-butyrobetaine is also a substrate and/or product for alpha-amioadipic semialdehyde dehydrogenase, aldehyde dehydrogenase (dimeric NADP-preferring), aldehyde dehydrogenase family 7 member A1, aldehyde dehydrogenase 1A3, aldehyde dehydrogenase (mitochondrial), fatty aldehyde dehydrogenase, aldehyde dehydrogenase X (mitochondrial) and 4-trimethylaminobutyraldehyde dehydrogenase.
Structure
Thumb
Synonyms
ValueSource
4-TrimethylammoniobutanoateChEBI
ButyrobetaineChEBI
gamma-ButyrobetaineChEBI
g-ButyrobetaineGenerator
γ-butyrobetaineGenerator
4-(N-trimethylamino)ButyrateHMDB
4-ButyrobetaineHMDB
ActinineHMDB
DeoxycarnitineHMDB
4-TrimethylaminobutyrateMeSH
Deoxy-carnitineMeSH
4-N-Trimethylammonium butyrateMeSH
Chemical FormulaC7H16NO2
Average Molecular Weight146.2074
Monoisotopic Molecular Weight146.118103761
IUPAC Name(3-carboxypropyl)trimethylazanium
Traditional Name4-trimethylaminobutyrate
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/p+1
InChI KeyJHPNVNIEXXLNTR-UHFFFAOYSA-O
Chemical Taxonomy
DescriptionThis compound 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
  • 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
  • Organic cation
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

  Organ and components:

Source:

  Biological:

    Animal:

Route of exposure:

  Enteral:

Process

Naturally occurring process:

  Biological process:

    Biochemical pathway:

    Cellular process:

Role

Biological role:

Industrial application:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.33 g/LALOGPS
logP-3ALOGPS
logP-4ChemAxon
logS-2.7ALOGPS
pKa (Strongest Acidic)4.46ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count2ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area37.3 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity51.44 m³·mol⁻¹ChemAxon
Polarizability16.63 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0a4i-9200000000-9e2d1dc212c339b92f26View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-0kmr-9210000000-6e4541ec3483e7b91d57View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-002b-1900000000-966adf9bec08be829132View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0ug1-7900000000-9bceb086039a8196b74eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9100000000-d347d928e06a35a20820View in MoNA
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Feces
  • Saliva
  • Urine
Tissue Location
  • Kidney
  • Liver
Pathways
NameSMPDB/PathwhizKEGG
Carnitine SynthesisPw000016Pw000016 greyscalePw000016 simpleNot Available
Displaying 1 entry
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified10.0 (0.5-20.0) uMAdult (>18 years old)BothNormal details
FecesDetected but not Quantified Infant (0-1 year old)Not Specified
Normal
details
FecesDetected but not Quantified Infant (0-1 year old)Not Specified
Normal
details
FecesDetected but not Quantified Infant (0-1 year old)Not AvailableNormal details
SalivaDetected and Quantified0.754 +/- 0.692 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified1.10 +/- 0.713 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
UrineExpected but not Quantified Not AvailableNot AvailableNormal
    details
    Abnormal Concentrations
    BiofluidStatusValueAgeSexConditionReferenceDetails
    FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
    Associated Disorders and Diseases
    Disease ReferencesNone
    Associated OMIM IDsNone
    DrugBank IDNot Available
    DrugBank Metabolite IDDBMET00521
    Phenol Explorer Compound IDNot Available
    Phenol Explorer Metabolite IDNot Available
    FoodDB IDFDB022456
    KNApSAcK IDNot Available
    Chemspider ID131
    KEGG Compound IDC01181
    BioCyc ID4-TRIMETHYLAMMONIOBUTANOATE
    BiGG ID53567
    Wikipedia LinkNot Available
    METLIN ID6043
    PubChem Compound134
    PDB IDNM2
    ChEBI ID1941
    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 ]

    Enzymes

    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
    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
    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
    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
    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
    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
    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