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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-11-09 23:15:21 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
Data?1582752180
Synonyms
ValueSource
3-DehydroxycarnitineChEBI
4-(N-Trimethylamino)butyrateChEBI
4-(Trimethylamino)butanoateChEBI
4-ButyrobetaineChEBI
ActinineChEBI
ButyrobetaineChEBI
DeoxycarnitineChEBI
gamma-ButyrobetainChEBI
gamma-ButyrobetaineChEBI
4-(N-Trimethylamino)butyric acidGenerator
4-(Trimethylamino)butanoic acidGenerator
g-ButyrobetainGenerator
Γ-butyrobetainGenerator
g-ButyrobetaineGenerator
Γ-butyrobetaineGenerator
4-TrimethylammoniobutanoateGenerator
Deoxy-carnitineHMDB
4-TrimethylaminobutyrateHMDB
4-N-Trimethylammonium butyrateHMDB
4-Trimethylammoniobutanoic acidHMDB
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
Disposition

Biological location

Process

Naturally occurring process

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
PropertyValueSource
Water Solubility0.33 g/LALOGPS
logP-3.2ALOGPS
logP-4ChemAxon
logS-3ALOGPS
pKa (Strongest Acidic)4.46ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count2ChemAxon
Hydrogen Donor Count0ChemAxon
Polar Surface Area40.13 ŲChemAxon
Rotatable Bond Count4ChemAxon
Refractivity62.28 m³·mol⁻¹ChemAxon
Polarizability16.2 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
AllCCS[M+H]+131.31632859911
AllCCS[M-H]-141.25332859911
DeepCCS[M+H]+130.07230932474
DeepCCS[M-H]-126.92330932474
DeepCCS[M-2H]-163.78230932474
DeepCCS[M+Na]+138.8130932474

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
4-Trimethylammoniobutanoic acidC[N+](C)(C)CCCC([O-])=O1562.9Standard polar33892256
4-Trimethylammoniobutanoic acidC[N+](C)(C)CCCC([O-])=O918.2Standard non polar33892256
4-Trimethylammoniobutanoic acidC[N+](C)(C)CCCC([O-])=O1067.3Semi standard non polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Predicted GC-MSPredicted GC-MS Spectrum - 4-Trimethylammoniobutanoic acid GC-MS (Non-derivatized) - 70eV, Positivesplash10-00di-9200000000-db9c27544fc4b78a3ffd2017-09-01Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 4-Trimethylammoniobutanoic acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid LC-ESI-QQ , positive-QTOFsplash10-0002-0900000000-859205a790bddd4399092017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid LC-ESI-QQ , positive-QTOFsplash10-000j-9500000000-2244afa7512434be351d2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid LC-ESI-QQ , positive-QTOFsplash10-000i-9000000000-2c1f817b20a8cbfba00c2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid LC-ESI-QQ , positive-QTOFsplash10-0006-9000000000-8c578b9dc166594a66be2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid LC-ESI-QQ , positive-QTOFsplash10-052e-9000000000-d1e5ff8334122de266a62017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid , positive-QTOFsplash10-000b-7900000000-ff7cb6ee0c5eec3fb8122017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 40V, Positive-QTOFsplash10-052e-9000000000-b4529cb8b31f26a2dc2a2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 10V, Positive-QTOFsplash10-0002-7900000000-990405988c934085cb3e2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 20V, Positive-QTOFsplash10-000i-9000000000-fa893a46940a677ca8842021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 35V, Positive-QTOFsplash10-000b-7900000000-f5baf5443f2ac35b90882021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 20V, Positive-QTOFsplash10-000i-9000000000-ceba93a4d1a8d5a358692021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 10V, Positive-QTOFsplash10-000b-9600000000-bc556243eb365d64176f2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 35V, Positive-QTOFsplash10-0002-5900000000-a1e724e53532d74ba5e72021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 35V, Positive-QTOFsplash10-000b-7900000000-c53120e4be9dd4abc3d72021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 20V, Negative-QTOFsplash10-0a4m-9300000000-0f1930134b0fc7910e122021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 40V, Negative-QTOFsplash10-001l-9000000000-73f9fa300c0d7955628b2021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 20V, Positive-QTOFsplash10-000m-9000000000-4f9fc35eb01531740b852021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 40V, Positive-QTOFsplash10-0005-9000000000-916262ee1da6f50e25022021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 10V, Negative-QTOFsplash10-0006-2900000000-0e3fd42c5d0320b3892c2021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 10V, Positive-QTOFsplash10-0f92-0900000000-543e3ddd695c95c84efd2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 20V, Positive-QTOFsplash10-0uds-6900000000-2935d3993a057a2a7fdd2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 40V, Positive-QTOFsplash10-0006-9100000000-1c7e8f5838068bd092fb2017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 10V, Negative-QTOFsplash10-0006-1900000000-f4142ed02e4763e047402017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 20V, Negative-QTOFsplash10-0006-5900000000-74df013a2a0033b163e12017-09-01Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 4-Trimethylammoniobutanoic acid 40V, Negative-QTOFsplash10-0kg6-9000000000-eb91f7fa144cb7eb7e802017-09-01Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
Biospecimen Locations
  • Blood
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Kidney
  • Liver
  • Placenta
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified10.0 (0.5-20.0) uMAdult (>18 years old)BothNormal details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Not Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Not Specified
Normal
details
FecesDetected but not QuantifiedNot QuantifiedInfant (0-1 year old)Not AvailableNormal details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
Normal
details
FecesDetected but not QuantifiedNot QuantifiedAdult (>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 QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
Normal
details
UrineExpected but not QuantifiedNot QuantifiedNot AvailableNot AvailableNormal
      Not Available
details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
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

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