| Common Name |
4-Trimethylammoniobutanoic acid
| Description |
4-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 |
MOLSDF3D-SDFPDBSMILESInChI View 3D Structure
| Synonyms |
| Value |
Source |
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 Formlia |
C7H16NO2
| Average Molecliar Weight |
146.2074
| Monoisotopic Molecliar Weight |
146.118103761
| IUPAC Name |
(3-carboxypropyl)trimethylazanium
| Traditional Name |
4-trimethylaminobutyrate
| CAS Registry Number |
407-64-7
| 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 Key |
JHPNVNIEXXLNTR-UHFFFAOYSA-O
| Chemical Taxonomy |
| Description |
This compound belongs to the class of organic compounds known as straight chain fatty acids. These are fatty acids with a straight aliphatic chain.
| Kingdom |
Organic compounds
| Super Class |
Lipids and lipid-like moleclies
| Class |
Fatty Acyls
| Sub Class |
Fatty acids and conjugates
| Direct Parent |
Straight chain fatty acids
| Alternative Parents |
Tetraalkylammonium salts
Monocarboxylic acids and derivatives
Carboxylic acids
Organopnictogen compounds
Organic salts
Organic oxides
Hydrocarbon derivatives
Carbonyl compounds
Amines
Organic cations
| 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
| Molecliar Framework |
Aliphatic acyclic compounds
| External Descriptors |
quaternary ammonium ion (CHEBI:1941 )
| Ontology |
| Status |
Detected 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 Emlisifiers
| Cellliar locations |
Cytoplasm
Extracellliar
Membrane
| Physical Properties |
| State |
Solid
| Experimental Properties |
| Property |
Value |
Reference |
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
| Predicted Properties |
| Property |
Value |
Source |
Water Solubility0.33 mg/mLALOGPS
logP-3ALOGPS
logP-4ChemAxon
logS-2.7ALOGPS
pKa (Strongest Acidic)4.46ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count2ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area37.3 Å2ChemAxon
Rotatable Bond Count4ChemAxon
Refractivity51.44 m3·mol-1ChemAxon
Polarizability16.63 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rlie of FiveYesChemAxon
Ghose FilterYesChemAxon
Vebers RlieYesChemAxon
MDDR-like RlieYesChemAxon
| Spectra |
| Spectra |
Not Available
| Biological Properties |
| Cellliar Locations |
Cytoplasm
Extracellliar
Membrane
| Biofluid Locations |
Blood
Feces
Saliva
Urine
| Tissue Location |
Kidney
Liver
| Pathways |
| Name |
SMPDB Link |
KEGG Link |
Carnitine SynthesisSMP00465Not Available
| Normal Concentrations |
| Biofluid |
Status |
Age |
Condition |
Reference |
Details |
BloodDetected and Quantified10.0 (0.5-20.0) uMAdlit (>18 years old)BothNormal
15164354
details
FecesDetected but not Quantified Infant (0-1 year old)Not AvailableNormal
24628373
details
SalivaDetected and Quantified0.754 +/- 0.692 uMAdlit (>18 years old)Not Specified
Normal
Sugimoto et al. (…
details
SalivaDetected and Quantified1.10 +/- 0.713 uMAdlit (>18 years old)Not Specified
Normal
Sugimoto et al. (…
details
SalivaDetected but not Quantified Adlit (>18 years old)Male
Normal
22308371
details
SalivaDetected but not Quantified Adlit (>18 years old)Male
Normal
22308371
details
SalivaDetected but not Quantified Adlit (>18 years old)Male
Normal
22308371
details
SalivaDetected but not Quantified Adlit (>18 years old)Male
Normal
22308371
details
SalivaDetected but not Quantified Adlit (>18 years old)Male
Normal
22308371
details
SalivaDetected but not Quantified Adlit (>18 years old)Male
Normal
22308371
details
SalivaDetected but not Quantified Adlit (>18 years old)Male
Normal
22308371
details
SalivaDetected but not Quantified Adlit (>18 years old)Male
Normal
22308371
details
SalivaDetected but not Quantified Adlit (>18 years old)Male
Normal
22308371
details
SalivaDetected but not Quantified Adlit (>18 years old)Male
Normal
22308371
details
UrineExpected but not Quantified Not AvailableNot AvailableNormal
details
|
| Abnormal Concentrations |
|
Not Available
| Associated Disorders and Diseases |
| Disease References |
None
| Associated OMIM IDs |
None
| External Links |
| DrugBank ID |
Not Available
| DrugBank Metabolite ID |
DBMET00521
| Phenol Explorer Compound ID |
Not Available
| Phenol Explorer Metabolite ID |
Not Available
| FoodDB ID |
FDB022456
| KNApSAcK ID |
Not Available
| Chemspider ID |
131
| KEGG Compound ID |
C01181
| BioCyc ID |
4-TRIMETHYLAMMONIOBUTANOATE
| BiGG ID |
53567
| Wikipedia Link |
Not Available
| NuGOwiki Link |
HMDB01161
| Metagene Link |
HMDB01161
| METLIN ID |
6043
| PubChem Compound |
134
| PDB ID |
NM2
| ChEBI ID |
1941
Product: JW74
References |
| Synthesis Reference |
Kawamura, 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 |
- 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 ]
- Siliprandi N, Ciman M, Sartorelli L: Myocardial carnitine transport. Basic Res Cardiol. 1987;82 Suppl 1:53-62. [PubMed:3311009 ]
- 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 ]
- 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 ]
- 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 + NADH |
details |
| 4-Trimethylammoniobutanal + NAD + Water → 4-Trimethylammoniobutanoic acid + NADH + Hydrogen Ion |
details |
- 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 Ion |
details |
- 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 Ion |
details |
- 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 Ion |
details |
- 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 Ion |
details |
- 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 dioxide |
details |
PMID: 17664946