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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-05-20 20:24:37 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-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. 4-Trimethylammoniobutanoic acid is also a substrate and/or product for alpha-aminoadipic 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

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Industrial application:

Biological role:

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
Biospecimen Locations
  • Blood
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Kidney
  • Liver
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
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
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
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
    BiospecimenStatusValueAgeSexConditionReferenceDetails
    FecesDetected but not Quantified Adult (>18 years old)Both
    Colorectal cancer
    details
    FecesDetected but not Quantified Adult (>18 years old)Both
    Colorectal cancer
    details
    FecesDetected but not Quantified Adult (>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
    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