iron(2+)

Three steps

• itaconyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 13 routes

• tetradecanoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 11 routes

• octanoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 4 moremitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. phytanate-CoA ligase

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 3 moremitochondrial matrix, cytosolOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 10 moremitochondrial matrix, cytosolOne of 15 routes

• prostaglandin H2Reach from here →
  1. De novo synthesis of 2Fe2S
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrixOne of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + AH2 = (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 7 moremitochondrial matrix

• sarcosineReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-Alanine:glyoxylate aminotransferase
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrixOne of 2 routes

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-Alanine:glyoxylate aminotransferase
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 7 moremitochondrial matrix

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and arginine (cytosol to extracellular)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosolOne of 6 routes

• carnosineReach from here →
  1. De novo synthesis of 2Fe2S
  2. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)
  3. beta-alanine + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, inner side of the membrane, outer side of the membrane

• 4-guanidinobutanoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and arginine (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosol

• L-dopaReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. L-dopa(out) + L-phenylalanine(in) = L-dopa(in) + L-phenylalanine(out)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(6)-(1,2-dicarboxyethyl)-AMPReach from here →
  1. De novo synthesis of 2Fe2S
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. IMP:L-aspartate ligase (GDP-forming)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrix, cytosolOne of 2 routes

• 3-guanidinopropanoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and arginine (cytosol to extracellular)
  3. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• L-histidineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. L-histidine(in) + L-phenylalanine(out) = L-histidine(out) + L-phenylalanine(in)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 7 routes

• D-ornithineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and ornithine (cytosol to extracellular)
  3. D-ornithine(in) + L-ornithine(out) = D-ornithine(out) + L-ornithine(in)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• an N-acylglycineReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-Alanine:glyoxylate aminotransferase
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 8 moremitochondrial matrix

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrixOne of 5 routes

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

  Catalysed by FECH, HMOX1, HMOX2, PIRmitochondrial matrix, cytosol

• 2-methyl-3-oxopropanoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 4 moremitochondrial matrix

• guanidinoacetateReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-Alanine:glyoxylate aminotransferase
  3. L-Arginine:glycine amidinotransferase

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• sphing-4-enineReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 7 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• spermidineReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, cytosolOne of 3 routes

• L-dopaquinoneReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,L-dopa:oxygen oxidoreductase

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosol

• glutathioneReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-Alanine:glyoxylate aminotransferase
  3. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrixOne of 2 routes

• L-saccharopineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and lysine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosol

• leukotriene C4Reach from here →
  1. De novo synthesis of 2Fe2S
  2. L-Alanine:glyoxylate aminotransferase
  3. leukotriene C4 hydrolysis

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, cytosol

• (4Z,15Z)-bilirubin IXalphaReach from here →
  1. Ferrochelatase, Mitochondrial
  2. Heme Oxygenase 1
  3. (4Z,15Z)-bilirubin IXalpha + NADP(+) = biliverdin IXalpha + NADPH + H(+)

  Catalysed by FECH, HMOX1, HMOX2, BLVRAmitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, cytosolOne of 2 routes

• taurocyamineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and arginine (cytosol to extracellular)
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosol

• L-glutamate 5-semialdehydeReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and ornithine (cytosol to extracellular)
  3. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosol

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach from here →
  1. De novo synthesis of 2Fe2S
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix

• L,L-cystathionineReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 4 moremitochondrial matrix

• gamma-L-glutamyl-L-cysteineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. L-glutamate:L-cysteine gamma-ligase (ADP-forming)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 6-amino-2-oxohexanoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and lysine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 4 moremitochondrial matrix, extracellular space, cytosol, peroxisome

• L-homocysteineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosol

• carbamoyl phosphateReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and glutamine (cytosol to extracellular)
  3. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 7 moremitochondrial matrix, extracellular space, cytosolOne of 4 routes

• S-methyl-L-methionineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosol

• glutathione disulfideReach from here →
  1. Fe(II):oxygen oxidoreductase
  2. cytochrome b reductase (ascorbate)
  3. glutathione:dehydroascorbate oxidoreductase

  Catalysed by CP, FTH1, FXN, FTMT and 2 moreextracellular space, cytosolOne of 2 routes

• L-tyrosineReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-Alanine:glyoxylate aminotransferase
  3. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrixOne of 4 routes

• an acyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 7 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• histamineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and histidine (cytosol to extracellular)
  3. L-histidine carboxy-lyase (histamine-forming)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosol

• L-2-aminopentanoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. 2-oxopentanoate + L-kynurenine = L-2-aminopentanoate + kynurenate + H2O

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrixOne of 2 routes

• (2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinateReach from here →
  1. De novo synthesis of 2Fe2S
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, cytosolOne of 2 routes

• kynurenateReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. Kynurenine-Oxoglutarate Transaminase

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrixOne of 13 routes

• L-2-aminohexanoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. 2-oxohexanoate + L-kynurenine = L-2-aminohexanoate + kynurenate + H2O

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrixOne of 2 routes

• 5-guanidino-2-oxopentanoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and arginine (cytosol to extracellular)
  3. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosol

• (3Z)-dodecenoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrixOne of 3 routes