homocysteine

Three steps

• 11-cis-retinolReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by CBS, NAGS, DGAT1, LRAT and 3 morecytosol, mitochondrial matrix

• cob(II)alaminReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. 2 cob(II)alamin + A + 2 H2O + 2 H(+) = 2 aquacob(III)alamin + AH2

  Catalysed by MTR, CDKAL1, MTRRcytosol

• (R)-3-aminoisobutyric acidReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by MTR, SLC7A5, AGXT2cytosol, extracellular space, mitochondrial matrix

• iodideReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

  Catalysed by MTR, SLC7A5, TPOcytosol, extracellular spaceOne of 34 routes

• oxaloacetateReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. ATP citrate lyase (citrate)

  Catalysed by CBS, NAGS, ACLYcytosol, mitochondrial matrixOne of 6 routes

• nitric oxideReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)

  Catalysed by MTR, SLC7A6, NOS3, NOS1 and 1 morecytosol, extracellular spaceOne of 3 routes

• CHEBI:16523Reach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. transport of D-serine and glycine (cytosol to extracellular)

  Catalysed by MTR, SLC7A5, SLC3A2, SLC7A10cytosol, extracellular spaceOne of 4 routes

• N(2)-acetyl-L-ornithineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and ornithine (cytosol to extracellular)
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by MTR, SLC7A6, ACY1cytosol, extracellular space

• 2-phenylacetamideReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by MTR, SLC7A5, EPXcytosol, extracellular space

• CHEBI:16633Reach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by MTR, SLC7A5, SCLYcytosol, extracellular space

• (S)-lactateReach from here →
  1. Formation of Homocystine
  2. transport of GSH and prostaglandin E2 (cytosol to extracellular)
  3. prostaglandin E2(out) + 2 (S)-lactate(in) = prostaglandin E2(in) + 2 (S)-lactate(out)

  Catalysed by SLCO1A2, SLCO1B3, SLCO2A1cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 14 routes

• 3'-deoxy-3',4'-didehydro-CTPReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. CTP + AH2 + S-adenosyl-L-methionine = 3'-deoxy-3',4'-didehydro-CTP + 5'-deoxyadenosine + L-methionine + A + H2O + H(+)

  Catalysed by MTR, CDKAL1, RSAD2cytosol

• taurochenodeoxycholate 3-sulfateReach from here →
  1. Formation of Homocystine
  2. solute carrier organic anion transporter (GSH)
  3. taurochenodeoxycholate + 3'-phosphoadenylyl sulfate = taurochenodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLCO1A2, SLC7A9, SLCO1B3, SULT2A1cytosol, extracellular space

• dopamine quinoneReach from here →
  1. Formation of Homocystine
  2. transport of dopamine and GSH (cytosol to extracellular)
  3. Peroxidase

  Catalysed by SLCO1A2, SLCO1B3, MPOcytosol, extracellular space, lysosomeOne of 4 routes

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and phenylalanine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by MTR, SLC7A5, CNDP2cytosol, extracellular space

• tryptamineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. L-tryptophan decarboxy-lyase

  Catalysed by MTR, SLC7A5, DDCcytosol, extracellular space

• 2-oxoglutaramateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by MTR, SLC7A5, KYAT1cytosol, extracellular spaceOne of 3 routes

• (S)-2-hydroxyglutarateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. (S)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2

  Catalysed by MTR, CDKAL1, L2HGDHcytosol

• glyoxylic acidReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by MTR, SLC7A5, DAOcytosol, extracellular space, peroxisome

• N-acetyl-L-aspartateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by MTR, SLC7A13, NAT8Lcytosol, inner side of the membrane, outer side of the membrane, mitochondrial matrixOne of 2 routes

• oxalic acidReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of chloride and methionine (cytosol to extracellular)
  3. transport of chloride and oxalate (cytosol to extracellular)

  Catalysed by MTR, SLC6A14, SLC26A6cytosol, extracellular space

• (R)-lactaldehydeReach from here →
  1. Formation of Homocystine
  2. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)
  3. (R)-Lactaldehyde:NAD+ oxidoreductase (glutathione-formylating)

  Catalysed by GLO1, GLOD4, ADH1C, ADH1A and 5 morecytosol

• L-glutamic 5-semialdehydeReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and ornithine (cytosol to extracellular)
  3. L-Ornithine:2-oxo-acid aminotransferase

  Catalysed by MTR, SLC7A6, OATcytosol, extracellular space, mitochondrial matrix

• dehydroascorbic acidReach from here →
  1. Formation of Homocystine
  2. transport of dopamine and GSH (cytosol to extracellular)
  3. dopamine,ascorbate:oxygen oxidoreductase (beta-hydroxylating)

  Catalysed by SLCO1A2, SLCO1B3, DBHcytosol, extracellular spaceOne of 19 routes

• anilineReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by CBS, NAGS, NAT2, NAT1cytosol, mitochondrial matrix

• CHEBI:17312Reach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by CBS, NAGScytosol, mitochondrial matrixOne of 2 routes

• 5'-deoxyadenosineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)

  Catalysed by MTR, CDKAL1, CDK5RAP1cytosolOne of 4 routes

• sulfiteReach from here →
  1. Formation of Homocystine
  2. Sulfide:glutathione,quinone oxidoreductase
  3. Persulfide dioxygenase

  Catalysed by SQOR, ETHE1cytosol, mitochondrial matrixOne of 2 routes

• CHEBI:17380Reach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by MTR, SLC7A5cytosol, extracellular spaceOne of 2 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by MTR, SLC7A5, PRODH2cytosol, extracellular spaceOne of 2 routes

• agmatineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by MTR, SLC7A6cytosol, extracellular space, mitochondrial matrix

• 5-aminolevulinic acidReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by MTR, SLC7A5, ALAS1, ALAS2 and 1 morecytosol, extracellular space, mitochondrial matrix

• indole-3-pyruvateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by MTR, SLC7A5, IL4I1cytosol, extracellular spaceOne of 2 routes

• 3,3',5-triiodo-L-thyronine sulfateReach from here →
  1. Formation of Homocystine
  2. transport of GSH and triiodothyronine (cytosol to extracellular)
  3. aryl sulfotransferase (PAPS)

  Catalysed by SLCO1A2, SLCO1B3, SULT1A3, SULT1A4 and 2 morecytosol, extracellular spaceOne of 2 routes

• L-thyroxine sulfateReach from here →
  1. Formation of Homocystine
  2. transport of GSH and thyroxine (cytosol to extracellular)
  3. L-thyroxine + 3'-phosphoadenylyl sulfate = L-thyroxine sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLCO1A2, SLCO1B3, SULT1A3, SULT1A4 and 1 morecytosol, extracellular space

• 3,3',5'-triiodo-L-thyronine sulfateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. 3,3',5'-triiodo-L-thyronine sulfate + iodide + A + H(+) = L-thyroxine sulfate + AH2

  Catalysed by MTR, CDKAL1, DIO1cytosol

• 3,3'-diiodo-L-thyronineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. 3,3'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine + AH2

  Catalysed by MTR, CDKAL1, DIO1, DIO3cytosolOne of 5 routes

• 3,3'-diiodo-L-thyronine sulfateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. 3,3'-diiodo-L-thyronine sulfate + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine sulfate + AH2

  Catalysed by MTR, CDKAL1, DIO1cytosolOne of 2 routes

• N-acetylserotoninReach from here →
  1. Formation of Homocystine
  2. transport of GSH and serotonin (cytosol to extracellular)
  3. acetyl-CoA:aralkylamine N-acetyltransferase

  Catalysed by SLCO1A2, SLCO1B3, AANATcytosol, extracellular space

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by CBS, NAGS, HADHA, ACADVL and 1 morecytosol, mitochondrial matrix

• Ala-tRNA(Ala)Reach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by MTR, SLC7A5, AARS1cytosol, extracellular space

• glycine betaineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of chloride and methionine (cytosol to extracellular)
  3. transport of betaine and chloride (cytosol to extracellular)

  Catalysed by MTR, SLC6A14, SLC6A12cytosol, extracellular spaceOne of 3 routes

• trans-urocanateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of histidine and methionine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by MTR, SLC7A6, HALcytosol, extracellular space

• urateReach from here →
  1. Formation of Homocystine
  2. glutathione:dehydroascorbate oxidoreductase
  3. ascorbate to monodehydroascorbate conversion

  No catalyst recordedcytosol

• 5-hydroxy-L-tryptophanReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin

  Catalysed by MTR, SLC7A5, TPH1, TPH2cytosol, extracellular spaceOne of 2 routes

• prostaglandin F1Reach from here →
  1. Formation of Homocystine
  2. transport of GSH and prostaglandin E1 (cytosol to extracellular)
  3. prostaglandin E1 + NADPH + H(+) = prostaglandin F1 + NADP(+)

  Catalysed by SLCO2A1, DHRS1cytosol, extracellular space

• 4-methyl-2-oxopentanoateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of leucine and methionine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by MTR, SLC3A2, SLC7A5, BCAT2cytosol, extracellular space, mitochondrial matrixOne of 4 routes

• CHEBI:17927Reach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by CBS, NAGScytosol, mitochondrial matrixOne of 2 routes

• 3-phenylpyruvateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and phenylalanine (cytosol to extracellular)
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by MTR, SLC7A5, GOT2, TATcytosol, extracellular space, mitochondrial matrixOne of 5 routes

• (R)-3,4-dihydroxymandelaldehydeReach from here →
  1. Formation of Homocystine
  2. transport of GSH and noradrenaline (cytosol to extracellular)
  3. (R)-noradrenaline + O2 + H2O = (R)-3,4-dihydroxymandelaldehyde + H2O2 + NH4(+)

  Catalysed by SLCO1A2, SLCO1B3, MAOA, MAOBcytosol, extracellular space