S-methyl-L-methionine

Three steps

• 3-hydroxyisovaleryl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by BHMT2, NAGS, CPT1A, CPT1Bmitochondrial matrix, cytosol

• CHEBI:63544Reach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• hexacosanoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• tetracosanoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 10 routes

• docosanoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 9 routes

• a 2,3-saturated acyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  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 BHMT2, CDKAL1, ACAD10

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

  Catalysed by BHMT2, SLC7A6, NOS3, NOS1 and 1 moreextracellular space, cytosol

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

  Catalysed by BHMT2, SLC7A5, MOCOSextracellular space, cytosol

• (2E)-octadecenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by BHMT2, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (DHA)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• heptadecanoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (2S)-2-aminobutanoateReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. transport of glycine and methionine (cytosol to extracellular)
  3. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine

  Catalysed by BHMT2, SLC7A5, AGXT2extracellular space, cytosolOne of 2 routes

• nonadecanoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by BHMT2, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Activation of dodecanedioic acid

  Catalysed by BHMT2, NAGSmitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Production of Sebacoylcarnitine

  Catalysed by BHMT2, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Production of Suberylcarnitine

  Catalysed by BHMT2, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by BHMT2, NAGSmitochondrial matrix, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by BHMT2, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by BHMT2, NAGS, CRAT, CROTmitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by BHMT2, NAGS, ACAA1, HSD17B4 and 2 moremitochondrial matrix, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Production of Hexadecanedioylcarnitine

  Catalysed by BHMT2, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 2 routes

• pristanoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. fatty-acid-CoA ligase

  Catalysed by BHMT2, NAGS, SLC27A2mitochondrial matrix, cytosolOne of 2 routes

• undecanoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by BHMT2, NAGS, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 3 routes

• (9S)-hydroxy-(10E,12Z)-octadecadienoateReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  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. (9Z,12Z)-octadecadienoate + AH2 + O2 = (9S)-hydroxy-(10E,12Z)-octadecadienoate + A + H2O

  Catalysed by BHMT2, CDKAL1, PTGS1, PTGS2

• (9R)-hydroxy-(10E,12Z)-octadecadienoateReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  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. (9Z,12Z)-octadecadienoate + AH2 + O2 = (9R)-hydroxy-(10E,12Z)-octadecadienoate + A + H2O

  Catalysed by BHMT2, CDKAL1, PTGS1, PTGS2

• (11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoateReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  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. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoate + A + H2O

  Catalysed by BHMT2, CDKAL1, PTGS2

• (15R)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  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. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (15R)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by BHMT2, CDKAL1, PTGS2

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

  Catalysed by BHMT2, SLC7A5, MOCOSextracellular space, cytosolOne of 2 routes

• glycodeoxycholateReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. transport of glycine and methionine (cytosol to extracellular)
  3. Glycodeoxycholate amidohydrolase

  Catalysed by BHMT2, SLC7A5extracellular space, cytosolOne of 2 routes

• (5Z)-tetradecenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (physeteric acid)

  Catalysed by BHMT2, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes

• (3E)-decenoyl-CoAReach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

  Catalysed by BHMT2, NAGS, ACADM, ECHS1 and 2 moremitochondrial matrix

• 2-glyceryl-prostaglandin H2Reach from here →
  1. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  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-glyceryl-prostaglandin G2 + AH2 = 2-glyceryl-prostaglandin H2 + A + H2O

  Catalysed by BHMT2, CDKAL1, PTGS2