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Record Information
Version4.0
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-09-27 08:07:59 UTC
HMDB IDHMDB0001325
Secondary Accession Numbers
  • HMDB01325
Metabolite Identification
Common NameN6,N6,N6-Trimethyl-L-lysine
DescriptionN6,N6,N6-Trimethyl-L-lysine is a methylated derivative of the amino acid lysine. It is a component of histone proteins, a precursor of carnitine and a coenzyme of fatty acid oxidation. N6,N6,N6-Trimethyl-L-lysine residues are found in a number of proteins and are generated by the action of S-adenosyl-L-methionine on exposed lysine residues. When trimethyllysine is released from cognate proteins via proteolysis, it serves as a precursor for carnitine biosynthesis. Mitochondrial 6-N-trimethyllysine dioxygenase converts 6-N-trimethyllysine to 3-hydroxy-6-N-trimethyllysine as the first step for carnitine biosynthesis. Because the subsequent carnitine biosynthesis enzymes are cytosolic, 3-hydroxy-6-N-trimethyllysine must be transported out of the mitochondria by a putative mitochondrial 6-N-trimethyllysine/3-hydroxy-6-N-trimethyllysine transporter system. Plasma -N-trimethyllysine concentrations are significantly lower in systemic carnitine deficiency patients compared to normal individuals, but no significant difference in urinary -N-trimethyllysine excretion is seen between the two groups.
Structure
Thumb
Synonyms
ValueSource
(S)-2-amino-6-(trimethylammonio)HexanoateHMDB
(S)-2-amino-6-(trimethylammonio)Hexanoic acidHMDB
delta-TrimethyllysineHMDB
epsilon-N-Trimethyl-L-lysineHMDB
epsilon-Trimethyl-L-lysineHMDB
N(6),N(6),N(6)-Trimethyl-L-lysineHMDB
S)-5-amino-5-Carboxy-N,N,N-trimethyl-1-pentanaminiumHMDB
TrimethyllysineHMDB
6-N-L-Trimethyl-L-lysineMeSH
epsilon-N-Trimethyl-lysineMeSH
TRIMETHYLLLYSINEMeSH
Trimethyllysine hydroxide, inner salt, (S)-isomerMeSH
Trimethyllysine, (+-)-isomerMeSH
Trimethyllysine hydroxide,inner salt, (+-)-isomerMeSH
Trimethyllysine chloride, (S)-isomerMeSH
Chemical FormulaC9H20N2O2
Average Molecular Weight188.2673
Monoisotopic Molecular Weight188.152477894
IUPAC Name(2S)-2-amino-6-(trimethylazaniumyl)hexanoate
Traditional Namen-trimethyllysine
CAS Registry Number19253-88-4
SMILES
C[N+](C)(C)CCCC[C@H](N)C([O-])=O
InChI Identifier
InChI=1S/C9H20N2O2/c1-11(2,3)7-5-4-6-8(10)9(12)13/h8H,4-7,10H2,1-3H3/t8-/m0/s1
InChI KeyMXNRLFUSFKVQSK-QMMMGPOBSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as l-alpha-amino acids. These are alpha amino acids which have the L-configuration of the alpha-carbon atom.
KingdomChemical entities
Super ClassOrganic compounds
ClassOrganic acids and derivatives
Sub ClassCarboxylic acids and derivatives
Direct ParentL-alpha-amino acids
Alternative Parents
Substituents
  • L-alpha-amino acid
  • Medium-chain fatty acid
  • Amino fatty acid
  • Fatty acyl
  • Fatty acid
  • Tetraalkylammonium salt
  • Quaternary ammonium salt
  • Carboxylic acid salt
  • Amino acid
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Amine
  • Organic zwitterion
  • Organic salt
  • Hydrocarbon derivative
  • Primary amine
  • Organooxygen compound
  • Organonitrogen compound
  • Organic oxide
  • Primary aliphatic amine
  • Organopnictogen compound
  • Organic oxygen compound
  • Carbonyl group
  • Organic nitrogen compound
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
Disposition

Biological Location:

  Subcellular:

  Biofluid and excreta:

Source:

Process

Naturally occurring process:

  Biological process:

    Biochemical pathway:

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 Solubility0.12 g/LALOGPS
logP-3.2ALOGPS
logP-6.2ChemAxon
logS-3.3ALOGPS
pKa (Strongest Acidic)2.41ChemAxon
pKa (Strongest Basic)9.53ChemAxon
Physiological Charge1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area66.15 ŲChemAxon
Rotatable Bond Count6ChemAxon
Refractivity74.63 m³·mol⁻¹ChemAxon
Polarizability21.55 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, PositiveNot AvailableView in JSpectraViewer
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-000l-0900000000-6306038d328c591e1128View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-01y7-2900000000-44354c8813190fe25a0eView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-08mi-9400000000-499e4dafbd41d754a57dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-000i-0900000000-3bc9f6f2c730e7f3785cView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-000i-2900000000-9928cba63f73f9126887View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-00di-9100000000-da0cc13b89df68e4b27fView in MoNA
Biological Properties
Cellular Locations
  • Cytoplasm
  • Endoplasmic reticulum
Biofluid Locations
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Location
  • Testes
Pathways
NameSMPDB/PathwhizKEGG
Carnitine SynthesisPw000016Pw000016 greyscalePw000016 simpleNot Available
Displaying 1 entry
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
Cerebrospinal Fluid (CSF)Detected and Quantified1.5 +/- 2.0 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified5.3 (3.8-10.4) umol/mmol creatinineAdult (>18 years old)MaleNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022556
KNApSAcK IDNot Available
Chemspider ID389120
KEGG Compound IDC03793
BioCyc IDN6N6N6-TRIMETHYL-L-LYSINE
BiGG ID42542
Wikipedia LinkNot Available
METLIN ID6161
PubChem Compound440120
PDB IDNot Available
ChEBI ID17311
References
Synthesis ReferenceChen F M; Benoiton N L A synthesis of N6,N6,N6-trimethyl-L-lysine dioxalate in gram amounts. Biochemistry and cell biology = Biochimie et biologie cellulaire (1986), 64(3), 182-3.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. 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 ]
  2. Inoue R, Miyake M, Kanazawa A, Sato M, Kakimoto Y: Decrease of 3-methylhistidine and increase of NG,NG-dimethylarginine in the urine of patients with muscular dystrophy. Metabolism. 1979 Aug;28(8):801-4. [PubMed:454517 ]
  3. Mizobuchi M, Miyake M, Sano A, Kakimoto Y: High concentration of free trimethyllysine in red blood cells. Biochim Biophys Acta. 1990 Feb 26;1033(2):119-23. [PubMed:2106342 ]
  4. Minkler PE, Erdos EA, Ingalls ST, Griffin RL, Hoppel CL: Improved high-performance liquid chromatographic method for the determination of 6-N,N,N-trimethyllysine in plasma and urine: biomedical application of chromatographic figures of merit and amine mobile phase modifiers. J Chromatogr. 1986 Aug 2;380(2):285-99. [PubMed:3093513 ]
  5. Lehman LJ, Olson AL, Rebouche CJ: Measurement of epsilon-N-trimethyllysine in human blood plasma and urine. Anal Biochem. 1987 Apr;162(1):137-42. [PubMed:3111294 ]
  6. Park KS, Lee HW, Hong SY, Shin S, Kim S, Paik WK: Determination of methylated amino acids in human serum by high-performance liquid chromatography. J Chromatogr. 1988 May 25;440:225-30. [PubMed:3136187 ]

Enzymes

General function:
Involved in peptidase activity
Specific function:
Component of the microsomal signal peptidase complex which removes signal peptides from nascent proteins as they are translocated into the lumen of the endoplasmic reticulum
Gene Name:
SPCS1
Uniprot ID:
Q9Y6A9
Molecular weight:
11804.7
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-N(6),N(6),N(6)-trimethyl-L-lysine + Succinic acid + CO(2)details
N6,N6,N6-Trimethyl-L-lysine + Oxoglutaric acid + Oxygen → 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine + Succinic acid + Carbon dioxidedetails