| Common Name |
N6,N6,N6-Trimethyl-L-lysine
| Description |
N6,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 |
MOLSDF3D-SDFPDBSMILESInChI View 3D Structure
| Synonyms |
| Value |
Source |
(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 Formlia |
C9H20N2O2
| Average Molecliar Weight |
188.2673
| Monoisotopic Molecliar Weight |
188.152477894
| IUPAC Name |
(2S)-2-amino-6-(trimethylazaniumyl)hexanoate
| Traditional Name |
n-trimethyllysine
| CAS Registry Number |
19253-88-4
| 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 Key |
MXNRLFUSFKVQSK-QMMMGPOBSA-N
| Chemical Taxonomy |
| Description |
This 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.
| Kingdom |
Chemical entities
| Super Class |
Organic compounds
| Class |
Organic acids and derivatives
| Sub Class |
Carboxylic acids and derivatives
| Direct Parent |
L-alpha-amino acids
| Alternative Parents |
Medium-chain fatty acids
Amino fatty acids
Tetraalkylammonium salts
Carboxylic acid salts
Amino acids
Monocarboxylic acids and derivatives
Carboxylic acids
Organopnictogen compounds
Organic zwitterions
Organic salts
Organic oxides
Monoalkylamines
Hydrocarbon derivatives
Carbonyl compounds
| 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
| Molecliar Framework |
Aliphatic acyclic compounds
| External Descriptors |
Not Available
| Ontology |
| Status |
Detected and Quantified
| Origin |
Endogenous
| Biofunction |
Protein synthesis, amino acid biosynthesis
| Application |
Not Available
| Cellliar locations |
Cytoplasm
Endoplasmic reticlium
| Physical Properties |
| State |
Solid
| Experimental Properties |
| Property |
Value |
Reference |
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
| Predicted Properties |
| Property |
Value |
Source |
Water Solubility0.12 mg/mLALOGPS
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 Å2ChemAxon
Rotatable Bond Count6ChemAxon
Refractivity74.63 m3·mol-1ChemAxon
Polarizability21.55 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rlie of FiveYesChemAxon
Ghose FilterYesChemAxon
Vebers RlieYesChemAxon
MDDR-like RlieYesChemAxon
| Spectra |
| Spectra |
Not Available
| Biological Properties |
| Cellliar Locations |
Cytoplasm
Endoplasmic reticlium
| Biofluid Locations |
Cerebrospinal Fluid (CSF)
Urine
| Tissue Location |
Testes
| Pathways |
| Name |
SMPDB Link |
KEGG Link |
Carnitine SynthesisSMP00465Not Available
| Normal Concentrations |
| Biofluid |
Status |
Age |
Condition |
Reference |
Details |
Cerebrospinal Fluid (CSF)Detected and Quantified1.5 +/- 2.0 uMAdlit (>18 years old)BothNormal
Geigy Scientific …
details
UrineDetected and Quantified5.3 (3.8-10.4) umol/mmol creatinineAdlit (>18 years old)MaleNormal
5472370
details
|
| Abnormal Concentrations |
|
Not Available
| Associated Disorders and Diseases |
| Disease References |
None
| Associated OMIM IDs |
None
| External Links |
| DrugBank ID |
Not Available
| DrugBank Metabolite ID |
Not Available
| Phenol Explorer Compound ID |
Not Available
| Phenol Explorer Metabolite ID |
Not Available
| FoodDB ID |
FDB022556
| KNApSAcK ID |
Not Available
| Chemspider ID |
389120
| KEGG Compound ID |
C03793
| BioCyc ID |
N6N6N6-TRIMETHYL-L-LYSINE
| BiGG ID |
42542
| Wikipedia Link |
Not Available
| NuGOwiki Link |
HMDB01325
| Metagene Link |
HMDB01325
| METLIN ID |
6161
| PubChem Compound |
440120
| PDB ID |
Not Available
| ChEBI ID |
17311
Product: Eupatilin
References |
| Synthesis Reference |
Chen 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 |
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 dioxide |
details |
PMID: 18198214