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3-HYDROXY-N6N6N6-TRIMETHYL-L-LYSINE BioCyc 1.0 C00318 KEGG Compound 937 ChemSpider P49189 UniProt S-Adenosylhomocysteine 439153 PubChem-compound SMILES O 131 ChemSpider Succinic acid 130 ChemSpider HMDB0001185 HMDB 50 ChemSpider 1.0 N6,N6,N6-Trimethyl-L-lysine 5962 PubChem-compound 64595-66-0 CAS 51 PubChem-compound Water Histone-lysine N-methyltransferase SETD7 CHEBI:15846 ChEBI C7H15NO3 L-Carnitine 161.1052 N6,N6,N6-Trimethyl-L-lysine 5747 ChemSpider Glycine C15H23N6O5S S-Adenosylmethionine 399.14505 BTO:0000759 BRENDA TISSUE ONTOLOGY 1038 PubChem-compound LYS BioCyc 952 ChemSpider 4-trimethylaminobutyraldehyde dehydrogenase Carbon dioxide 274 ChemSpider 4-trimethylaminobutyraldehyde dehydrogenase C5H6O5 Oxoglutaric acid 146.02153 CHEBI:16908 ChEBI 1.0 Serine hydroxymethyltransferase, cytosolic ADENOSYL-HOMO-CYS BioCyc Serine hydroxymethyltransferase, cytosolic 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine 485-80-3 CAS 133 PubChem-compound 134 PubChem-compound 1010 ChemSpider SMILES C[N+](C)(C)CCCC(O)[C@H](N)C(O)=O L-Lysine 4.0 Mitochondrion GLY BioCyc ReactionCatalysis199 ACTIVATION 1.0 ReactionCatalysis197 ACTIVATION ReactionCatalysis195 ACTIVATION ReactionCatalysis196 ACTIVATION 541-15-1 CAS N6,N6,N6-Trimethyl-L-lysine SubPathwayInput SMILES C[S+](CC[C@H](N)C(O)=O)C[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2N 979-92-0 CAS HMDB0000208 HMDB CPD-6641 BioCyc C14H20N6O5S S-Adenosylhomocysteine 384.12158 SUC BioCyc 58-68-4 CAS HMDB0001422 HMDB 56-87-1 CAS SMILES OC(=O)CCC(=O)C(O)=O Serine hydroxymethyltransferase, cytosolic 4.0 CHEBI:30915 ChEBI 124-38-9 CAS C2H5NO2 Glycine 75.03203 C00007 KEGG Compound 388565 ChemSpider CHEBI:15414 ChEBI C00001 KEGG Compound C00004 KEGG Compound C00003 KEGG Compound Liver 1.0 5257127 PubChem-compound GO:0005634 GENE ONTOLOGY Oxygen C00019 KEGG Compound CHEBI:15741 ChEBI C21H28N7O14P2 NAD 664.11694 S-ADENOSYLMETHIONINE BioCyc C00011 KEGG Compound SMILES O=C=O 1.0 HMDB0000902 HMDB N6N6N6-TRIMETHYL-L-LYSINE BioCyc Oxygen 1110 PubChem-compound C9H20N2O2 N6,N6,N6-Trimethyl-L-lysine 188.15248 C21H29N7O14P2 NADH 665.12476 HMDB0001325 HMDB 1.0 Histone-lysine N-methyltransferase SETD7 CHEBI:1941 ChEBI C4H6O4 Succinic acid 118.02661 H Hydrogen Ion 1.007825 388301 ChemSpider CARNITINE BioCyc SMILES NCCCC[C@H](N)C(O)=O C00021 KEGG Compound 53-84-9 CAS C00026 KEGG Compound HMDB0000123 HMDB GO:0005739 GENE ONTOLOGY Nucleoplasm GO:0005737 GENE ONTOLOGY Gamma-butyrobetaine dioxygenase C7H16NO 4-Trimethylammoniobutanal 130.12318 1.0 1.0 NADH SMILES NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1O Trimethyllysine dioxygenase, mitochondrial C00037 KEGG Compound 5682 ChemSpider 328-50-7 CAS SMILES NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O SMILES C[N+](C)(C)CCCC[C@H](N)C([O-])=O HMDB0000254 HMDB Trimethyllysine dioxygenase, mitochondrial SMILES OC(=O)CCC(O)=O 1.0 HMDB0001345 HMDB 1.0 4-Trimethylammoniobutanoic acid BiologicalState31 Homo sapiens, Liver Gamma-butyrobetaine dioxygenase 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine Transport15 false 4-Trimethylammoniobutanoic acid (→) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Liver, Cell, Cytoplasm LEFT_TO_RIGHT C00042 KEGG Compound C03793 KEGG Compound C01259 KEGG Compound 1078 ChemSpider C00047 KEGG Compound 1.0 HMDB0000939 HMDB Carbon dioxide 4-Trimethylammoniobutanal C9H21N2O3 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine 205.15521 10917 PubChem-compound SMILES C[N+](C)(C)CCCC=O 1.0 Cytoplasm 1.0 10455 ChemSpider ReactionCatalysis212 ACTIVATION CHEBI:18019 ChEBI CHEBI:15786 ChEBI Oxoglutaric acid Q9NVH6 UniProt Homo sapiens C01149 KEGG Compound 7732-18-5 CAS 388299 ChemSpider 25203146 PubChem-compound HMDB0001487 HMDB Reaction130 false 4-Trimethylammoniobutanal + NAD + Water → 4-Trimethylammoniobutanoic acid + Hydrogen Ion + NADH LEFT_TO_RIGHT Hydrogen Ion O2 Oxygen 31.98983 SubPathwayInteraction69 SubPathway69Reaction SubPathwayReaction 1.14.11.1 false 1.14.11.1 4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen → Carbon dioxide + L-Carnitine + Succinic acid LEFT_TO_RIGHT C7H16NO2 4-Trimethylammoniobutanoic acid 146.1181 CHEBI:16526 ChEBI P34896 UniProt 56-40-6 CAS CHEBI:18020 ChEBI 7782-44-7 CAS 4-trimethylaminobutyraldehyde dehydrogenase HMDB0001377 HMDB SMILES C[N+](C)(C)C[C@H](O)CC([O-])=O 1.0 CHEBI:15428 ChEBI GO:0005654 GENE ONTOLOGY Carnitine Synthesis 9606 TAXONOMY 962 PubChem-compound 31983 ChemSpider 2.0 2-KETOGLUTARATE BioCyc 389120 ChemSpider 1.0 Trimethyllysine dioxygenase, mitochondrial CO2 Carbon dioxide 43.98983 5893 PubChem-compound C00080 KEGG Compound SMILES O=O 110-15-6 CAS 730 ChemSpider Oxoglutaric acid HMDB0002111 HMDB CHEBI:15377 ChEBI CHEBI:15379 ChEBI CHEBI:16347 ChEBI C6H14N2O2 L-Lysine 146.10553 19253-88-4 CAS CHEBI:15378 ChEBI SMILES [H+] O75936 UniProt PW000016 PathWhiz SMILES C[N+](C)(C)CCCC(O)=O Protein degradation SubPathway 1.0 S-Adenosylmethionine 1.0 CHEBI:17311 ChEBI C01181 KEGG Compound 280 PubChem-compound 1.0 HMDB0000062 HMDB HMDB0000182 HMDB 4-TRIMETHYLAMMONIOBUTANAL BioCyc 1.0 1.0 L-Carnitine SMILES N[C@@H](CCSC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2N)C(O)=O 25246222 PubChem-compound 407-64-7 CAS 440120 PubChem-compound H2O Water 18.010565 4-TRIMETHYLAMMONIOBUTANOATE BioCyc Succinic acid SMILES NCC(O)=O SMP0000465 SMPDB 1.14.11.8 false 1.14.11.8 N6,N6,N6-Trimethyl-L-lysine + Oxoglutaric acid + Oxygen → 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine + Carbon dioxide + Succinic acid LEFT_TO_RIGHT 2.1.2.1 false 2.1.2.1 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine → 4-Trimethylammoniobutanal + Glycine LEFT_TO_RIGHT 2.1.1.43 false 2.1.1.43 L-Lysine + S-Adenosylmethionine → N6,N6,N6-Trimethyl-L-lysine + S-Adenosylhomocysteine LEFT_TO_RIGHT HMDB0001161 HMDB NADH BioCyc Nucleus NAD BioCyc 977 PubChem-compound HMDB0059597 HMDB HMDB0001967 HMDB CHEBI:16680 ChEBI 1.0 24762165 PubChem-compound 1.0 Q8WTS6 UniProt NAD N6,N6,N6-Trimethyl-L-lysine SubPathwayOutput Histone-lysine N- methyltransferase SETD7 Trimethyllysine dioxygenase, mitochondrial Serine hydroxymethyltransferase, cytosolic 4- trimethylaminobutyraldehyde dehydrogenase Gamma- butyrobetaine dioxygenase N6,N6,N6-Trimethyl-L-lysine N6,N6,N6-Trimethyl-L-lysine 4-Trimethylammoniobutanoic acid 4-Trimethylammoniobutanoic acid L-Lysine S-Adenosylmethionine S-Adenosylhomocysteine Oxoglutaric acid O 2 3-Hydroxy-N6,N6,N6- trimethyl-L-lysine Succinic acid CO 2 Glycine 4-Trimethylammoniobutanal NAD H 2 O NADH H + Oxoglutaric acid O 2 L-Carnitine Succinic acid CO 2 Fe2+ Pyridoxal 5'-phosphate Fe2+ Ascorbic acid Protein containing a lysine residue Protein containing a lysine residue Protein degradation Nucleus Nuclear Membrane Nucleoplasm Extacellular Space Intracellular Space Intracellular Space Mitochondria Liver Kidney
3-HYDROXY-N6N6N6-TRIMETHYL-L-LYSINE BioCyc 1.0 C00318 KEGG Compound 937 ChemSpider P49189 UniProt S-Adenosylhomocysteine 439153 PubChem-compound SMILES O 131 ChemSpider Succinic acid 130 ChemSpider HMDB0001185 HMDB 50 ChemSpider 1.0 N6,N6,N6-Trimethyl-L-lysine 5962 PubChem-compound 64595-66-0 CAS 51 PubChem-compound Water Histone-lysine N-methyltransferase SETD7 CHEBI:15846 ChEBI C7H15NO3 L-Carnitine 161.1052 N6,N6,N6-Trimethyl-L-lysine 5747 ChemSpider Glycine C15H23N6O5S S-Adenosylmethionine 399.14505 BTO:0000759 BRENDA TISSUE ONTOLOGY 1038 PubChem-compound LYS BioCyc 952 ChemSpider 4-trimethylaminobutyraldehyde dehydrogenase Carbon dioxide 274 ChemSpider 4-trimethylaminobutyraldehyde dehydrogenase C5H6O5 Oxoglutaric acid 146.02153 CHEBI:16908 ChEBI 1.0 Serine hydroxymethyltransferase, cytosolic ADENOSYL-HOMO-CYS BioCyc Serine hydroxymethyltransferase, cytosolic 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine 485-80-3 CAS 133 PubChem-compound 134 PubChem-compound 1010 ChemSpider SMILES C[N+](C)(C)CCCC(O)[C@H](N)C(O)=O L-Lysine 4.0 Mitochondrion GLY BioCyc ReactionCatalysis199 ACTIVATION 1.0 ReactionCatalysis197 ACTIVATION ReactionCatalysis195 ACTIVATION ReactionCatalysis196 ACTIVATION 541-15-1 CAS N6,N6,N6-Trimethyl-L-lysine SubPathwayInput SMILES C[S+](CC[C@H](N)C(O)=O)C[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2N 979-92-0 CAS HMDB0000208 HMDB CPD-6641 BioCyc C14H20N6O5S S-Adenosylhomocysteine 384.12158 SUC BioCyc 58-68-4 CAS HMDB0001422 HMDB 56-87-1 CAS SMILES OC(=O)CCC(=O)C(O)=O Serine hydroxymethyltransferase, cytosolic 4.0 CHEBI:30915 ChEBI 124-38-9 CAS C2H5NO2 Glycine 75.03203 C00007 KEGG Compound 388565 ChemSpider CHEBI:15414 ChEBI C00001 KEGG Compound C00004 KEGG Compound C00003 KEGG Compound Liver 1.0 5257127 PubChem-compound GO:0005634 GENE ONTOLOGY Oxygen C00019 KEGG Compound CHEBI:15741 ChEBI C21H28N7O14P2 NAD 664.11694 S-ADENOSYLMETHIONINE BioCyc C00011 KEGG Compound SMILES O=C=O 1.0 HMDB0000902 HMDB N6N6N6-TRIMETHYL-L-LYSINE BioCyc Oxygen 1110 PubChem-compound C9H20N2O2 N6,N6,N6-Trimethyl-L-lysine 188.15248 C21H29N7O14P2 NADH 665.12476 HMDB0001325 HMDB 1.0 Histone-lysine N-methyltransferase SETD7 CHEBI:1941 ChEBI C4H6O4 Succinic acid 118.02661 H Hydrogen Ion 1.007825 388301 ChemSpider CARNITINE BioCyc SMILES NCCCC[C@H](N)C(O)=O C00021 KEGG Compound 53-84-9 CAS C00026 KEGG Compound HMDB0000123 HMDB GO:0005739 GENE ONTOLOGY Nucleoplasm GO:0005737 GENE ONTOLOGY Gamma-butyrobetaine dioxygenase C7H16NO 4-Trimethylammoniobutanal 130.12318 1.0 1.0 NADH SMILES NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1O Trimethyllysine dioxygenase, mitochondrial C00037 KEGG Compound 5682 ChemSpider 328-50-7 CAS SMILES NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O SMILES C[N+](C)(C)CCCC[C@H](N)C([O-])=O HMDB0000254 HMDB Trimethyllysine dioxygenase, mitochondrial SMILES OC(=O)CCC(O)=O 1.0 HMDB0001345 HMDB 1.0 4-Trimethylammoniobutanoic acid BiologicalState31 Homo sapiens, Liver Gamma-butyrobetaine dioxygenase 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine Transport15 false 4-Trimethylammoniobutanoic acid (→) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Liver, Cell, Cytoplasm LEFT_TO_RIGHT C00042 KEGG Compound C03793 KEGG Compound C01259 KEGG Compound 1078 ChemSpider C00047 KEGG Compound 1.0 HMDB0000939 HMDB Carbon dioxide 4-Trimethylammoniobutanal C9H21N2O3 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine 205.15521 10917 PubChem-compound SMILES C[N+](C)(C)CCCC=O 1.0 Cytoplasm 1.0 10455 ChemSpider ReactionCatalysis212 ACTIVATION CHEBI:18019 ChEBI CHEBI:15786 ChEBI Oxoglutaric acid Q9NVH6 UniProt Homo sapiens C01149 KEGG Compound 7732-18-5 CAS 388299 ChemSpider 25203146 PubChem-compound HMDB0001487 HMDB Reaction130 false 4-Trimethylammoniobutanal + NAD + Water → 4-Trimethylammoniobutanoic acid + Hydrogen Ion + NADH LEFT_TO_RIGHT Hydrogen Ion O2 Oxygen 31.98983 SubPathwayInteraction69 SubPathway69Reaction SubPathwayReaction 1.14.11.1 false 1.14.11.1 4-Trimethylammoniobutanoic acid + Oxoglutaric acid + Oxygen → Carbon dioxide + L-Carnitine + Succinic acid LEFT_TO_RIGHT C7H16NO2 4-Trimethylammoniobutanoic acid 146.1181 CHEBI:16526 ChEBI P34896 UniProt 56-40-6 CAS CHEBI:18020 ChEBI 7782-44-7 CAS 4-trimethylaminobutyraldehyde dehydrogenase HMDB0001377 HMDB SMILES C[N+](C)(C)C[C@H](O)CC([O-])=O 1.0 CHEBI:15428 ChEBI GO:0005654 GENE ONTOLOGY Carnitine Synthesis 9606 TAXONOMY 962 PubChem-compound 31983 ChemSpider 2.0 2-KETOGLUTARATE BioCyc 389120 ChemSpider 1.0 Trimethyllysine dioxygenase, mitochondrial CO2 Carbon dioxide 43.98983 5893 PubChem-compound C00080 KEGG Compound SMILES O=O 110-15-6 CAS 730 ChemSpider Oxoglutaric acid HMDB0002111 HMDB CHEBI:15377 ChEBI CHEBI:15379 ChEBI CHEBI:16347 ChEBI C6H14N2O2 L-Lysine 146.10553 19253-88-4 CAS CHEBI:15378 ChEBI SMILES [H+] O75936 UniProt PW000016 PathWhiz SMILES C[N+](C)(C)CCCC(O)=O Protein degradation SubPathway 1.0 S-Adenosylmethionine 1.0 CHEBI:17311 ChEBI C01181 KEGG Compound 280 PubChem-compound 1.0 HMDB0000062 HMDB HMDB0000182 HMDB 4-TRIMETHYLAMMONIOBUTANAL BioCyc 1.0 1.0 L-Carnitine SMILES N[C@@H](CCSC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C1N=CN=C2N)C(O)=O 25246222 PubChem-compound 407-64-7 CAS 440120 PubChem-compound H2O Water 18.010565 4-TRIMETHYLAMMONIOBUTANOATE BioCyc Succinic acid SMILES NCC(O)=O SMP0000465 SMPDB 1.14.11.8 false 1.14.11.8 N6,N6,N6-Trimethyl-L-lysine + Oxoglutaric acid + Oxygen → 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine + Carbon dioxide + Succinic acid LEFT_TO_RIGHT 2.1.2.1 false 2.1.2.1 3-Hydroxy-N6,N6,N6-trimethyl-L-lysine → 4-Trimethylammoniobutanal + Glycine LEFT_TO_RIGHT 2.1.1.43 false 2.1.1.43 L-Lysine + S-Adenosylmethionine → N6,N6,N6-Trimethyl-L-lysine + S-Adenosylhomocysteine LEFT_TO_RIGHT HMDB0001161 HMDB NADH BioCyc Nucleus NAD BioCyc 977 PubChem-compound HMDB0059597 HMDB HMDB0001967 HMDB CHEBI:16680 ChEBI 1.0 24762165 PubChem-compound 1.0 Q8WTS6 UniProt NAD N6,N6,N6-Trimethyl-L-lysine SubPathwayOutput Nucleus SETD7 TMLHE SHMT1 ALDH9A1 BBOX1 N6,N6,N6- Trimethyl-L- lysine N6,N6,N6- Trimethyl-L- lysine 4- Trimethylammoniobutanoic acid 4- Trimethylammoniobutanoic acid L-Lysine S- Adenosylmethionine S- Adenosylhomocysteine Oxoglutaric acid Oxygen 3-Hydroxy- N6,N6,N6- trimethyl-L- lysine Succinic acid Carbon dioxide Glycine 4- Trimethylammoniobutanal NAD Water NADH Hydrogen Ion Oxoglutaric acid Oxygen L-Carnitine Succinic acid Carbon dioxide Protein containing a lysine residue Protein containing a lysine residue Protein degradation