heme b

Three steps

• glycineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. L-Alanine:glyoxylate aminotransferase

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 7 morecytosol, mitochondrial matrixOne of 9 routes

• presqualene diphosphateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. heme b + (2E,6E)-farnesyl diphosphate + H2O = Fe(II)-heme o + diphosphate
  3. farnesyl-diphosphate:farnesyl-diphosphate farnesyltransferase

  Catalysed by COX10, FDFT1mitochondrial matrix, cytosolOne of 3 routes

• prostaglandin H2Reach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O

  Catalysed by COX10, COX15, PTGS1, PTGS2mitochondrial matrixOne of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + AH2 = (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by COX10, COX15, TBXAS1, PTGS2 and 1 moremitochondrial matrix

• D-alanineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-(3,4-dihydroxybenzoyloxy)-4,6-dihydroxybenzoateReach from here →
  1. Heme Oxygenase 1
  2. quercetin + O2 = 2-(3,4-dihydroxybenzoyloxy)-4,6-dihydroxybenzoate + CO
  3. quercetin + O2 = 2-(3,4-dihydroxybenzoyloxy)-4,6-dihydroxybenzoate + CO

  Catalysed by HMOX1, HMOX2, PIRcytosol

• D-threonineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-methionineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. transport of alanine and methionine (cytosol to extracellular)

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix, extracellular spaceOne of 11 routes

• L-tryptophanReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. transport of alanine and tryptophan (cytosol to extracellular)

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix, extracellular spaceOne of 4 routes

• L-threonineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 4 routes

• L-kynurenineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix

• beta-alanineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-asparagineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. transport of alanine and asparagine (cytosol to extracellular)

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix, extracellular spaceOne of 6 routes

• (2E)-geranyl diphosphateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. heme b + (2E,6E)-farnesyl diphosphate + H2O = Fe(II)-heme o + diphosphate
  3. isopentenyl diphosphate + (2E)-geranyl diphosphate = (2E,6E)-farnesyl diphosphate + diphosphate

  Catalysed by COX10, GGPS1, FDPSmitochondrial matrix

• L-phenylalanineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. transport of alanine and phenylalanine (cytosol to extracellular)

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix, extracellular spaceOne of 6 routes

• 3-hydroxy-L-kynurenineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix

• N(omega),N(omega)-dimethyl-L-arginineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix

• trans-4-hydroxy-L-prolineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• all-trans-nonaprenyl diphosphateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. heme b + (2E,6E)-farnesyl diphosphate + H2O = Fe(II)-heme o + diphosphate
  3. 6 isopentenyl diphosphate + (2E,6E)-farnesyl diphosphate = all-trans-nonaprenyl diphosphate + 6 diphosphate

  Catalysed by COX10, PDSS1, PDSS2mitochondrial matrix

• (2E,6E,10E)-geranylgeranyl diphosphateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. heme b + (2E,6E)-farnesyl diphosphate + H2O = Fe(II)-heme o + diphosphate
  3. Farnesyltranstransferase

  Catalysed by COX10, GGPS1mitochondrial matrix, cytosolOne of 2 routes

• 5-methylsulfanyl-2,3-dioxopentyl phosphateReach from here →
  1. Heme Oxygenase 1
  2. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 3-(methylsulfanyl)propanoate + CO + formate + 2 H(+)
  3. 5-methylsulfanyl-2,3-dioxopentyl phosphate + H2O = 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + phosphate

  Catalysed by HMOX1, HMOX2, ADI1, ENOPH1cytosol

• monodehydro-L-ascorbate radicalReach from here →
  1. Heme Oxygenase 1
  2. Fe(II):oxygen oxidoreductase
  3. Fe(3+)(out) + L-ascorbate(in) = monodehydro-L-ascorbate radical(in) + Fe(2+)(out) + H(+)

  Catalysed by HMOX1, HMOX2, CP, FTH1 and 8 morecytosol, extracellular space, inner side of the membrane, outer side of the membrane

• L-prolineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• all-trans-decaprenyl diphosphateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. heme b + (2E,6E)-farnesyl diphosphate + H2O = Fe(II)-heme o + diphosphate
  3. decaprenyl diphosphate synthase (farnesyl-PP)

  Catalysed by COX10, PDSS1, PDSS2mitochondrial matrix, cytosolOne of 2 routes

• (2E,6E)-farnesyl triphosphateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. heme b + (2E,6E)-farnesyl diphosphate + H2O = Fe(II)-heme o + diphosphate
  3. (2E,6E)-farnesyl diphosphate + ATP = (2E,6E)-farnesyl triphosphate + ADP

  Catalysed by COX10, NME1mitochondrial matrix

• L-cysteinylglycineReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. gamma-glutamyltransferase (alanine)

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 11 morecytosol, mitochondrial matrixOne of 2 routes

• a 2,3-saturated acyl-CoAReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. a 2,3-saturated acyl-CoA + A = a 2,3-dehydroacyl-CoA + AH2

  Catalysed by COX10, COX15, ACAD10mitochondrial matrix

• Mo-molybdopterinReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix

• (2S)-2-aminobutanoateReach from here →
  1. Heme Oxygenase 1
  2. De novo synthesis of 2Fe2S
  3. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate

  Catalysed by HMOX1, HMOX2, NDUFAB1, FDXR and 6 morecytosol, mitochondrial matrix

• (9S)-hydroxy-(10E,12Z)-octadecadienoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (9Z,12Z)-octadecadienoate + AH2 + O2 = (9S)-hydroxy-(10E,12Z)-octadecadienoate + A + H2O

  Catalysed by COX10, COX15, PTGS1, PTGS2mitochondrial matrix

• (9R)-hydroxy-(10E,12Z)-octadecadienoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (9Z,12Z)-octadecadienoate + AH2 + O2 = (9R)-hydroxy-(10E,12Z)-octadecadienoate + A + H2O

  Catalysed by COX10, COX15, PTGS1, PTGS2mitochondrial matrix

• (11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoate + A + H2O

  Catalysed by COX10, COX15, PTGS2mitochondrial matrix

• (15R)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (15R)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by COX10, COX15, PTGS2mitochondrial matrix

• thio-Mo-molybdopterinReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by COX10, COX15, MOCOSmitochondrial matrix

• 2-glyceryl-prostaglandin H2Reach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. 2-glyceryl-prostaglandin G2 + AH2 = 2-glyceryl-prostaglandin H2 + A + H2O

  Catalysed by COX10, COX15, PTGS2mitochondrial matrix

• (2E,6E)-farnesyl phosphateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. heme b + (2E,6E)-farnesyl diphosphate + H2O = Fe(II)-heme o + diphosphate
  3. (2E,6E)-farnesyl diphosphate + H2O = (2E,6E)-farnesyl phosphate + phosphate + H(+)

  Catalysed by COX10, NUDT18, PLPP6, NUDT15mitochondrial matrix

• prostaglandin H1Reach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. prostaglandin G1 + AH2 = prostaglandin H1 + A + H2O

  Catalysed by COX10, COX15, PTGS2mitochondrial matrix

• (5S,11R)-dihydroxy-(6E,8Z,12E,14Z)-eicosatetraenoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (5S,11R)-dihydroxy-(6E,8Z,12E,14Z)-eicosatetraenoate + A + H2O

  Catalysed by COX10, COX15, PTGS2mitochondrial matrix

• (5S,15R)-dihydroxy-(6E,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (5S,15R)-dihydroxy-(6E,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by COX10, COX15, PTGS2mitochondrial matrix

• (5S,15S)-dihydroxy-(6E,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (5S,15S)-dihydroxy-(6E,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by COX10, COX15, PTGS2mitochondrial matrix

• 17R-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate + AH2 + O2 = 17R-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoate + A + H2O

  Catalysed by COX10, COX15, PTGS2mitochondrial matrix

• 13-hydroxy-(4Z,7Z,10Z,14E,16Z,19Z)-docosahexaenoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate + AH2 + O2 = 13-hydroxy-(4Z,7Z,10Z,14E,16Z,19Z)-docosahexaenoate + A + H2O

  Catalysed by COX10, COX15, PTGS2mitochondrial matrix

• (18R)-hydroxy-(5Z,8Z,11Z,14Z,16E)-eicosapentaenoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoate + AH2 + O2 = (18R)-hydroxy-(5Z,8Z,11Z,14Z,16E)-eicosapentaenoate + A + H2O

  Catalysed by COX10, COX15, PTGS2mitochondrial matrix

• (15R)-hydroxy-(5Z,8Z,11Z,13E,17Z)-eicosapentaenoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoate + AH2 + O2 = (15R)-hydroxy-(5Z,8Z,11Z,13E,17Z)-eicosapentaenoate + A + H2O

  Catalysed by COX10, COX15, PTGS2mitochondrial matrix

• (11R)-hydroxy-(5Z,8Z,12E,14Z,17Z)-eicosapentaenoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoate + AH2 + O2 = (11R)-hydroxy-(5Z,8Z,12E,14Z,17Z)-eicosapentaenoate + A + H2O

  Catalysed by COX10, COX15, PTGS2mitochondrial matrix

• 13R-hydroxy-(7Z,10Z,14E,16Z,19Z)-docosapentaenoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (7Z,10Z,13Z,16Z,19Z)-docosapentaenoate + AH2 + O2 = 13R-hydroxy-(7Z,10Z,14E,16Z,19Z)-docosapentaenoate + A + H2O

  Catalysed by COX10, COX15, PTGS2mitochondrial matrix

• (13S)-hydroxy-(9Z,11E)-octadecadienoateReach from here →
  1. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  2. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2
  3. (9Z,12Z)-octadecadienoate + AH2 + O2 = (13S)-hydroxy-(9Z,11E)-octadecadienoate + A + H2O

  Catalysed by COX10, COX15, PTGS1, PTGS2mitochondrial matrix