homocysteine

Three steps

• L-homoarginineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. L-homoarginine(in) + L-arginine(out) = L-homoarginine(out) + L-arginine(in)

  Catalysed by MTR, SLC7A6, SLC25A29cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 4 routes

• L-homocarnosineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of histidine and methionine (cytosol to extracellular)
  3. Formation of homocarnosine

  Catalysed by MTR, SLC7A6, CARNS1cytosol, extracellular space

• 2',3'-cGAMPReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)
  3. 2',3'-cGAMP(out) + 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamide(in) = 2',3'-cGAMP(in) + 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamide(out)

  Catalysed by MTR, ATIC, SLC19A1cytosol, inner side of the membrane, outer side of the membrane

• N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. 5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)

  Catalysed by MTR, SLC7A5, GARTcytosol, extracellular space

• L-cystine-bis-glycineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

  Catalysed by MTR, SLC7A5, DPEP1, DPEP2cytosol, extracellular spaceOne of 2 routes

• glycyldehydrophenylalanineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

  Catalysed by MTR, SLC7A5, DPEP1cytosol, extracellular space

• 8-hydroperoxy-(5Z,9E,11Z,14Z)-eicosatetraenoateReach from here →
  1. Formation of Homocystine
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. arachidonate to 8-peroxy-(5Z,9E,11Z,14Z)-eicosatetraenoate conversion

  No catalyst recordedcytosol, extracellular spaceOne of 2 routes

• 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + L-glutamine + ATP + H2O = 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + L-glutamate + ADP + phosphate + H(+)

  Catalysed by MTR, SLC7A5, PFAScytosol, extracellular space

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

  Catalysed by MTR, SLC7A5, CMA1cytosol, extracellular spaceOne of 3 routes

• N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-serineReach from here →
  1. Formation of Homocystine
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-serine + H2O = (5Z,8Z,11Z,14Z)-eicosatetraenoate + L-serine

  Catalysed by FAAH, PM20D1cytosol, extracellular space

• an N-(fatty acyl)-glycineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

  Catalysed by MTR, SLC7A5, GLYATL2cytosol, extracellular space

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

  Catalysed by MTR, SLC7A5, GLYATL2cytosol, extracellular space

• (7Z)-octadecenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)

  Catalysed by CBS, NAGS, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 3 routes

• (15S)-15-hydroperoxy-prostaglandin E2Reach from here →
  1. Formation of Homocystine
  2. transport of GSH and prostaglandin G2 (cytosol to extracellular)
  3. prostaglandin G2 = (15S)-15-hydroperoxy-prostaglandin E2

  Catalysed by SLCO2A1, PTGEScytosol, extracellular space

• acetaldehydeReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and threonine (cytosol to extracellular)
  3. L-threonine acetaldehyde-lyase (glycine-forming)

  Catalysed by MTR, SLC7A5cytosol, extracellular space

• CHEBI:15345Reach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Acetoacetate:CoA ligase (AMP-forming)

  Catalysed by CBS, NAGS, AACScytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by CBS, NAGS, CHATcytosol, mitochondrial matrix, nucleusOne of 7 routes

• pyruvateReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. L-cystathionine cysteine-lyase (deaminating
  3. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate

  Catalysed by CBS, CTH, AGXT2cytosolOne of 17 routes

• 5,6,7,8-tetrahydrobiopterinReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. histamine N-methyltransferase (SAM)
  3. 5-methyl-THF to 5,10-methylene-THF conversion

  Catalysed by MTR, HNMTcytosol

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by MTR, SLC7A5, TPH1, TPH2cytosol, extracellular spaceOne of 3 routes

• CHEBI:15380Reach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by CBS, NAGS, ALAS1, CPNE7cytosol, mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by CBS, NAGS, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• 2-methylcrotonoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Production of Tiglylcarnitine

  Catalysed by CBS, NAGS, CPT1A, CPT1Bcytosol, mitochondrial matrixOne of 2 routes

• isobutyryl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by CBS, NAGS, HADHA, ACAA2 and 1 morecytosol, mitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by CBS, NAGS, GLYATcytosol, mitochondrial matrix

• isovaleryl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by CBS, NAGS, GLYAT, GLYATL2 and 1 morecytosol, mitochondrial matrixOne of 3 routes

• arachidonoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Arachidonate:CoA ligase (AMP-forming)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 14 routes

• CHEBI:15519Reach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. propanoyl-CoA C-acyltransferase

  Catalysed by CBS, NAGS, ACAA1, SCP2 and 1 morecytosol, mitochondrial matrix, peroxisomeOne of 3 routes

• palmitoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Palmitate:CoA ligase (AMP-forming)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 11 routes

• oleoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by CBS, NAGS, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 11 routes

• phenylacetyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Phenylacetate:CoA ligase

  Catalysed by CBS, NAGScytosol, mitochondrial matrixOne of 3 routes

• propionyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. L-cystathionine cysteine-lyase (deaminating
  3. 2-oxobutyrate oxidation

  Catalysed by CBS, CTHcytosolOne of 44 routes

• CHEBI:15570Reach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. transport of D-alanine and glycine (cytosol to extracellular)

  Catalysed by MTR, SLC7A5, SLC3A2, SLC7A10cytosol, extracellular spaceOne of 5 routes

• 12(S)-HPETEReach from here →
  1. Formation of Homocystine
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. arachidonate:oxygen 12-oxidoreductase

  Catalysed by ALOX12cytosol, extracellular spaceOne of 4 routes

• 15(S)-HPETEReach from here →
  1. Formation of Homocystine
  2. Glutathione: 5-HPETE oxidoreductase
  3. Glutathione: 15-HPETE oxidoreductase

  Catalysed by GPX5, GPX1, GPX2, GPX3 and 5 morecytosolOne of 6 routes

• 5(S)-HPETEReach from here →
  1. Formation of Homocystine
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. Arachidonate:oxygen 5-oxidoreductase

  Catalysed by ALOX5cytosol, extracellular space

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach 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

• trimethylenediamineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of lysine and methionine (cytosol to extracellular)
  3. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by MTR, SLC7A6, DHPScytosol, extracellular space

• CHEBI:15727Reach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)

  Catalysed by CBS, NAGS, NAA10cytosol, mitochondrial matrixOne of 2 routes

• formateReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. dimethylglycine dehydrogenase (formaldehyde)
  3. formaldehyde + NAD(+) + H2O = formate + NADH + 2 H(+)

  Catalysed by BHMT, BHMT2, PDPR, DMGDH and 1 morecytosolOne of 2 routes

• CHEBI:15746Reach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by CBS, NAGS, SAT1, SAT2cytosol, mitochondrial matrixOne of 2 routes

• CHEBI:15765Reach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of methionine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

  Catalysed by MTR, SLC7A5, THcytosol, extracellular spaceOne of 5 routes

• N-carbamoyl-L-aspartic acidReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. carbamoyl-phosphate:L-aspartate carbamoyltransferase

  Catalysed by MTR, SLC7A13, CADcytosol, inner side of the membrane, outer side of the membrane

• alpha-D-glucosamine 6-phosphateReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

  Catalysed by MTR, SLC7A5, GFPT2, GFPT1cytosol, extracellular space

• thioredoxin dithiolReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. L-Methionine:tRNAMet ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by MTR, MARS1cytosolOne of 2 routes

• (R)-lactateReach from here →
  1. Formation of Homocystine
  2. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)
  3. (R)-S-lactoylglutathione + H2O = (R)-lactate + glutathione + H(+)

  Catalysed by GLO1, GLOD4, HAGHcytosol

• methanethiolReach from here →
  1. Trimethylaminoacetate:L-homosysteine S-methyltransferase
  2. dimethylglycine dehydrogenase (formaldehyde)
  3. methanethiol + O2 + H2O = hydrogen sulfide + formaldehyde + H2O2 + H(+)

  Catalysed by BHMT, BHMT2, PDPR, DMGDH and 1 morecytosol

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

  Catalysed by MTR, SLC6A14, SLC26A6cytosol, extracellular spaceOne of 4 routes

• ureaReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. L-Arginine amidinohydrolase

  Catalysed by MTR, SLC7A6, ARG1cytosol, extracellular spaceOne of 3 routes

• L-cystineReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. L-cystathionine cysteine-lyase (deaminating
  3. thioredoxin-disulfide reductase (NADPH) (cystine)

  Catalysed by CBS, CTH, TXNRD1, TXNRD3 and 1 morecytosolOne of 10 routes