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Record Information
Version4.0
StatusExpected but not Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2019-07-23 05:44:58 UTC
HMDB IDHMDB0001422
Secondary Accession Numbers
  • HMDB01422
Metabolite Identification
Common Name3-Hydroxy-N6,N6,N6-trimethyl-L-lysine
Description3-Hydroxy-N6,N6,N6-trimethyl-L-lysine is found within the Lysine degration pathway; it is the byproduct of trimethyllysine dioxygenase (EC 1.14.11.8) which converts N6-Hydroxy-trimethyl-lysine to Glycine and 4-Trimethyl-ammoniobutanal. Because carnitine is the final product of the Lysine degration pathway, this compound is often associated with carnitine defiency disorders. (PMID: 16707092 ) The compound is also a byproduct of glycine hydroxymethyltransferase (EC 2.1.2.1) and Lyases (EC 4.1.2.-).
Structure
Data?1563860698
Synonyms
ValueSource
3-Hydroxy-N(6),N(6),N(6)-trimethyl-L-lysineHMDB
3-Hydroxy-N(6),N(6),N(6)-trimethyllysineHMDB
3-Hydroxy-N6,N6,N6-trimethyl-L-lysin-N6-iumHMDB
Chemical FormulaC9H21N2O3
Average Molecular Weight205.2746
Monoisotopic Molecular Weight205.155217548
IUPAC Name[(5S)-5-amino-5-carboxy-4-hydroxypentyl]trimethylazanium
Traditional Name[(5S)-5-amino-5-carboxy-4-hydroxypentyl]trimethylazanium
CAS Registry NumberNot Available
SMILES
C[N+](C)(C)CCCC(O)[C@H](N)C(O)=O
InChI Identifier
InChI=1S/C9H20N2O3/c1-11(2,3)6-4-5-7(12)8(10)9(13)14/h7-8,12H,4-6,10H2,1-3H3/p+1/t7?,8-/m0/s1
InChI KeyZRJHLGYVUCPZNH-MQWKRIRWSA-O
Chemical Taxonomy
Description belongs to the class of organic compounds known as l-alpha-amino acids. These are alpha amino acids which have the L-configuration of the alpha-carbon atom.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentL-alpha-amino acids
Alternative Parents
Substituents
  • L-alpha-amino acid
  • Medium-chain hydroxy acid
  • Medium-chain fatty acid
  • Amino fatty acid
  • Beta-hydroxy acid
  • Hydroxy fatty acid
  • Hydroxy acid
  • Fatty acid
  • Fatty acyl
  • Tetraalkylammonium salt
  • Quaternary ammonium salt
  • Amino acid
  • Secondary alcohol
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Hydrocarbon derivative
  • Amine
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Primary aliphatic amine
  • Organic oxide
  • Organopnictogen compound
  • Alcohol
  • Organic salt
  • Carbonyl group
  • Organic oxygen compound
  • Organic nitrogen compound
  • Organic cation
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Disposition

Biological location:

Source:

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 Solubility1.83 g/LALOGPS
logP-2.9ALOGPS
logP-7.1ChemAxon
logS-2.1ALOGPS
pKa (Strongest Acidic)2.2ChemAxon
pKa (Strongest Basic)8.99ChemAxon
Physiological Charge1ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count3ChemAxon
Polar Surface Area83.55 ŲChemAxon
Rotatable Bond Count6ChemAxon
Refractivity65.15 m³·mol⁻¹ChemAxon
Polarizability22.75 ųChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectrum TypeDescriptionSplash KeyView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-05dl-9700000000-76e306d0df20cdf2383dJSpectraViewer | MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (2 TMS) - 70eV, Positivesplash10-0ff0-9851000000-e9d6504a39fd334e4b9cJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-052r-0910000000-120b9ce241c6f49b2ac9JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-0006-0900000000-27173a0a79897e2f7ac9JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0w2l-9600000000-912689a68e2339f3eb51JSpectraViewer | MoNA
Biological Properties
Cellular Locations
  • Mitochondria
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 IDFDB022613
KNApSAcK IDNot Available
Chemspider ID388565
KEGG Compound IDC01259
BioCyc IDNot Available
BiGG ID37211
Wikipedia LinkNot Available
METLIN ID6234
PubChem Compound439460
PDB IDNot Available
ChEBI ID15786
Food Biomarker OntologyNot Available
VMH IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. van Vlies N, Wanders RJ, Vaz FM: Measurement of carnitine biosynthesis enzyme activities by tandem mass spectrometry: differences between the mouse and the rat. Anal Biochem. 2006 Jul 1;354(1):132-9. Epub 2006 May 2. [PubMed:16707092 ]

Enzymes

General function:
Involved in oxidation reduction
Specific function:
Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML).
Gene Name:
TMLHE
Uniprot ID:
Q9NVH6
Molecular weight:
44048.895
Reactions
N6,N6,N6-Trimethyl-L-lysine + Oxoglutaric acid + Oxygen → 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine + Succinic acid + Carbon dioxidedetails