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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2014-07-02 15:18:25 UTC
HMDB IDHMDB01161
Secondary Accession NumbersNone
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
  1. 4-(N-Trimethylamino)butyrate
  2. 4-Butyrobetaine
  3. 4-Trimethylammoniobutanoate
  4. 4-Trimethylammoniobutanoic acid
  5. Actinine
  6. Butyrobetaine
  7. Deoxycarnitine
  8. gamma-Butyrobetaine
Chemical FormulaC7H16NO2
Average Molecular Weight146.2074
Monoisotopic Molecular Weight146.118103761
IUPAC Name(3-carboxypropyl)trimethylazanium
Traditional IUPAC Name(3-carboxypropyl)trimethylazanium
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
KingdomOrganic Compounds
Super ClassLipids
ClassFatty Acids and Conjugates
Sub ClassAmino Fatty Acids
Other Descriptors
  • Aliphatic Acyclic Compounds
  • Organic Compounds
  • Straight Chain Fatty Acids
  • quaternary ammonium ion(ChEBI)
Substituents
  • Carboxylic Acid
  • Quaternary Ammonium Salt
Direct ParentAmino Fatty Acids
Ontology
StatusDetected and Quantified
Origin
  • Drug metabolite
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Fuel and energy storage
  • Fuel or energy source
  • Lipid catabolism, Fatty acid transport, Energy production
  • Membrane integrity/stability
  • Waste products
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Cytoplasm
  • Extracellular
  • Membrane
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 area37.3ChemAxon
rotatable bond count4ChemAxon
refractivity51.44ChemAxon
polarizability16.63ChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biofluid Locations
  • Blood
  • Urine
Tissue Location
  • Kidney
  • Liver
Pathways
NameSMPDB LinkKEGG Link
Carnitine SynthesisSMP00465Not Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified10.0 (0.5-20.0) uMAdult (>18 years old)BothNormal details
UrineExpected but not QuantifiedNot ApplicableNot AvailableNot AvailableNormal
  • Not Applicable
details
Abnormal Concentrations
Not Available
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
NuGOwiki LinkHMDB01161
Metagene LinkHMDB01161
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