homocysteine

Three steps

• leukotriene D4Reach from here →
  1. Formation of Homocystine
  2. leukotriene C4 synthase (GSH)
  3. leukotriene C4 + H2O = leukotriene D4 + L-glutamate

  Catalysed by LTC4S, GGT1, GGT5cytosol

• dTMPReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5,10-Methylenetetrahydrofolate:dUMP C-methyltransferase

  Catalysed by MTR, SHMT1, TYMScytosol

• CHEBI:63544Reach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes

• 18-hydroxy-(5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. Formation of Homocystine
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. cytochrome P450 (arachidonate)

  Catalysed by CYP4F8cytosol, extracellular space, endoplasmic reticulumOne of 2 routes

• hexacosanoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• tetracosanoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 10 routes

• docosanoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 9 routes

• a 2,3-saturated acyl-CoAReach 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. a 2,3-saturated acyl-CoA + A = a 2,3-dehydroacyl-CoA + AH2

  Catalysed by MTR, CDKAL1, ACAD10cytosol

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of arginine and methionine (cytosol to extracellular)
  3. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by MTR, SLC7A6, NOS3, NOS1 and 1 morecytosol, extracellular space

• Mo-molybdopterinReach from here →
  1. 5-methyltetrahydrofolate:L-homocysteine S-methyltransferase
  2. transport of alanine and methionine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by MTR, SLC7A5, MOCOScytosol, extracellular space

• (R)-adrenalineReach from here →
  1. Formation of Homocystine
  2. transport of GSH and noradrenaline (cytosol to extracellular)
  3. (R)-noradrenaline + S-adenosyl-L-methionine = (R)-adrenaline + S-adenosyl-L-homocysteine + H(+)

  Catalysed by SLCO1A2, SLCO1B3, PNMTcytosol, extracellular space

• (2E)-octadecenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Carnitine O-Palmitoyltransferase

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

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 8 routes

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

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (DPA)

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

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

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

• (11Z)-eicosenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

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

• (17Z)-hexacosenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 4 routes

• CHEBI:742324Reach from here →
  1. Formation of Homocystine
  2. transport of dopamine and GSH (cytosol to extracellular)
  3. S-Adenosyl-L-methionine:catechol O-methyltransferase

  Catalysed by SLCO1A2, SLCO1B3, COMT, LRTOMTcytosol, extracellular space

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (DHA)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes

• heptadecanoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (margaric acid)

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

• (9Z)-heptadecenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• (2S)-2-aminobutanoateReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. L-cystathionine cysteine-lyase (deaminating
  3. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O

  Catalysed by CBS, CTH, AADATcytosolOne of 7 routes

• nonadecanoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

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

• (11Z)-octadecenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

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

• (12R)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoateReach from here →
  1. Formation of Homocystine
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + O2 = (12R)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate

  Catalysed by ALOX12Bcytosol, extracellular space

• (8S)-hydroperoxy-(5Z,9E,11Z,14Z)-eicosatetraenoateReach from here →
  1. Formation of Homocystine
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. Arachidonate:oxygen 8-oxidoreductase

  Catalysed by ALOX12cytosol, extracellular spaceOne of 2 routes

• 2-O-(5Z,8Z,11Z,14Z)-eicosatetraenylglycerolReach from here →
  1. Formation of Homocystine
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. fatty acid amide hydrolase (arachidonate)

  Catalysed by FAAHcytosol, extracellular space

• dodecanedioyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Activation of dodecanedioic acid

  Catalysed by CBS, NAGScytosol, mitochondrial matrixOne of 4 routes

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

  Catalysed by CBS, NAGS, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 4 routes

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

  Catalysed by CBS, NAGS, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by CBS, NAGScytosol, mitochondrial matrixOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• 2-iminobutanoateReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. L-cystathionine cysteine-lyase (deaminating
  3. 2-iminobutanoate + H2O = 2-oxobutanoate + NH4(+)

  Catalysed by CBS, CTH, RIDAcytosol

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by CBS, NAGS, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrix

• (13Z)-eicosenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 5 routes

• 11,12-epoxy-(5Z,8Z,14Z)-eicosatrienoateReach from here →
  1. Formation of Homocystine
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + reduced [NADPH--hemoprotein reductase] + O2 = 11,12-epoxy-(5Z,8Z,14Z)-eicosatrienoate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by CYP2C9cytosol, extracellular space

• 19-hydroxy-(5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. Formation of Homocystine
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + reduced [NADPH--hemoprotein reductase] + O2 = 19-hydroxy-(5Z,8Z,11Z,14Z)-eicosatetraenoate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by CYP1A1, CYP1A2, CYP2C9, CYP2C19 and 1 morecytosol, extracellular space

• 20-oxo-(5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. Formation of Homocystine
  2. Resistance-Nodulation-Cell Division (Rnd) Tcdb:2.A.60.1.2
  3. 10-HETE[n] + NAD+[n] = H+[n] + NADH[n] + 20-oxo-(5Z,8Z,11Z,14Z)-eicosatetraenoate[n]

  Catalysed by SLCO2A1, ADH4cytosol, extracellular space, nucleusOne of 3 routes

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by CBS, NAGS, CRAT, CROTcytosol, mitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. L-serine hydro-lyase (adding homocysteine
  2. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by CBS, NAGS, ACAA1, HSD17B4 and 2 morecytosol, mitochondrial matrix, peroxisome