You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Version4.0
StatusExpected but not Quantified
Creation Date2008-08-12 16:51:29 UTC
Update Date2017-12-07 01:59:49 UTC
HMDB IDHMDB0006831
Secondary Accession Numbers
  • HMDB06831
Metabolite Identification
Common Name3-Dehydroxycarnitine
Description3-Dehydroxycarnitine is an acylcarnitine. Numerous disorders have been described that lead to disturbances in energy production and in intermediary metabolism in the organism 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, its impaired reabsorption by the kidney and, consequently, in increased urinary loss of L-carnitine. 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 physico-chemical 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
Thumb
Synonyms
ValueSource
4-(N-trimethylamino)ButyrateChEBI
4-ButyrobetaineChEBI
ActinineChEBI
ButyrobetaineChEBI
DeoxycarnitineChEBI
gamma-ButyrobetainChEBI
gamma-ButyrobetaineChEBI
4-(N-trimethylamino)Butyric acidGenerator
g-ButyrobetainGenerator
γ-butyrobetainGenerator
g-ButyrobetaineGenerator
γ-butyrobetaineGenerator
Deoxy-carnitineMeSH
4-TrimethylaminobutyrateMeSH
4-N-Trimethylammonium butyrateMeSH
Chemical FormulaC7H15NO2
Average Molecular Weight145.1995
Monoisotopic Molecular Weight145.110278729
IUPAC Name4-(trimethylazaniumyl)butanoate
Traditional Namebutyrobetaine
CAS Registry NumberNot Available
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
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
  • 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

Route of exposure:

Biological location:

Source:

Process

Naturally occurring process:

Role

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 Solubility0.22 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
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, Positivesplash10-00di-9200000000-db9c27544fc4b78a3ffdView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-0002-0900000000-859205a790bddd439909View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-000j-9500000000-2244afa7512434be351dView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-000i-9000000000-2c1f817b20a8cbfba00cView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-0006-9000000000-8c578b9dc166594a66beView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , positivesplash10-052e-9000000000-d1e5ff8334122de266a6View in MoNA
LC-MS/MSLC-MS/MS Spectrum - , positivesplash10-000b-7900000000-ff7cb6ee0c5eec3fb812View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-0f92-0900000000-543e3ddd695c95c84efdView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0uds-6900000000-2935d3993a057a2a7fddView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9100000000-1c7e8f5838068bd092fbView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0006-1900000000-f4142ed02e4763e04740View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0006-5900000000-74df013a2a0033b163e1View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0kg6-9000000000-eb91f7fa144cb7eb7e80View in MoNA
Biological Properties
Cellular LocationsNot Available
Biospecimen LocationsNot Available
Tissue LocationsNot Available
Pathways
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB024107
KNApSAcK IDNot Available
Chemspider ID705
KEGG Compound IDC05543
BioCyc IDGAMMA-BUTYROBETAINE
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN IDNot Available
PubChem Compound725
PDB IDNot Available
ChEBI ID16244
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. 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 ]

Enzymes

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