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Record Information
Version4.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2019-07-23 05:44:53 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
Data?1563860693
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-lysineHMDB
epsilon-N-Trimethyl-lysineHMDB
TRIMETHYLLLYSINEHMDB
Trimethyllysine hydroxide, inner salt, (S)-isomerHMDB
Trimethyllysine, (+-)-isomerHMDB
Trimethyllysine hydroxide,inner salt, (+-)-isomerHMDB
Trimethyllysine chloride, (S)-isomerHMDB
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
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 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

Source:

Biological location:

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
Spectrum TypeDescriptionSplash KeyView
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0abc-9300000000-17fb5edf4cea1b4dcafdJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-000l-0900000000-6306038d328c591e1128JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-01y7-2900000000-44354c8813190fe25a0eJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-08mi-9400000000-499e4dafbd41d754a57dJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-000i-0900000000-3bc9f6f2c730e7f3785cJSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-000i-2900000000-9928cba63f73f9126887JSpectraViewer | MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-00di-9100000000-da0cc13b89df68e4b27fJSpectraViewer | MoNA
Biological Properties
Cellular Locations
  • Cytoplasm
  • Endoplasmic reticulum
Biospecimen Locations
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Urine
Tissue Locations
  • Testes
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
Cerebrospinal Fluid (CSF)Detected and Quantified1.5 +/- 2.0 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
UrineDetected and Quantified5.3 (3.8-10.4) umol/mmol creatinineAdult (>18 years old)MaleNormal details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
FecesDetected but not Quantified Adult (>18 years old)Both
Colorectal cancer
details
Associated Disorders and Diseases
Disease References
Colorectal cancer
  1. 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 ]
  2. 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
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. 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 ]
  2. 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 ]
  3. 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 ]
  4. 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 ]
  5. 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 ]
  6. 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 ]

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