iron(2+)

Three steps

• heptadecanoyl-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 (margaric acid)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z)-heptadecenoyl-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 (9-heptadecylenic acid)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-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 ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 5 routes

• nonadecanoyl-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 (nonadecylic acid)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

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

• dodecanedioyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Activation of dodecanedioic acid

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 3 moremitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Production of Sebacoylcarnitine

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

• octanedioyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Production of Suberylcarnitine

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

• hexanedioyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 3 moremitochondrial matrix, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-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 ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-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 ((11Z,14Z)-eicosadienoic acid)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 5 routes

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrix, cytosol

• (13Z)-eicosenoyl-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 ((13Z)-eicosenoic acid)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 5 routes

• 2-aminoprop-2-enoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)
  3. D-serine = 2-aminoprop-2-enoate + H2O

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

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

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

• tetradecanedioyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

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

• hexadecanedioyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Production of Hexadecanedioylcarnitine

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

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

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 4 moremitochondrial matrix, cytosolOne of 2 routes

• undecanoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 9 moremitochondrial matrix, cytosolOne of 3 routes

• (9S)-hydroxy-(10E,12Z)-octadecadienoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (9Z,12Z)-octadecadienoate + AH2 + O2 = (9S)-hydroxy-(10E,12Z)-octadecadienoate + A + H2O

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrix

• (9R)-hydroxy-(10E,12Z)-octadecadienoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (9Z,12Z)-octadecadienoate + AH2 + O2 = (9R)-hydroxy-(10E,12Z)-octadecadienoate + A + H2O

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrix

• (11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoateReach 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 + O2 = (11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoate + A + H2O

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

• (15R)-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. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (15R)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O

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

• thio-Mo-molybdopterinReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

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

• glycodeoxycholateReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-Alanine:glyoxylate aminotransferase
  3. Glycodeoxycholate amidohydrolase

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

• (R)-lipoateReach from here →
  1. Fe(II):oxygen oxidoreductase
  2. cytochrome b reductase (ascorbate)
  3. ascorbate to dehydroascorbic acid conversion

  Catalysed by CP, FTH1, FXN, FTMT and 2 moreextracellular space, cytosol

• (5Z)-tetradecenoyl-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 (physeteric acid)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 8 routes

• (3E)-decenoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

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

• 2-glyceryl-prostaglandin H2Reach from here →
  1. De novo synthesis of 2Fe2S
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 2-glyceryl-prostaglandin G2 + AH2 = 2-glyceryl-prostaglandin H2 + A + H2O

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

• N-tetradecanoylglycineReach from here →
  1. Fe(II):oxygen oxidoreductase
  2. Fe(3+)(out) + L-ascorbate(in) = monodehydro-L-ascorbate radical(in) + Fe(2+)(out) + H(+)
  3. N-tetradecanoylglycine + 2 L-ascorbate + O2 = N-tetradecanoyl-(2S)-hydroxyglycine + 2 monodehydro-L-ascorbate radical + H2O

  Catalysed by CP, FTH1, FXN, FTMT and 7 moreextracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrix

• N-eicosanoylglycinateReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-Alanine:glyoxylate aminotransferase
  3. eicosanoyl-CoA + glycine = N-eicosanoylglycinate + CoA + H(+)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrix

• N-docosanoylglycineReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-Alanine:glyoxylate aminotransferase
  3. docosanoyl-CoA + glycine = N-docosanoylglycine + CoA + H(+)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrix

• N-hexacosanoylglycineReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-Alanine:glyoxylate aminotransferase
  3. hexacosanoyl-CoA + glycine = N-hexacosanoylglycine + CoA + H(+)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrix

• an N(2)-acyl-L-glutamineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and glutamine (cytosol to extracellular)
  3. an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 8 moremitochondrial matrix, extracellular space, cytosol

• (7Z)-hexadecenoyl-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 (7-palmitoleic acid)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 8 routes

• tricosanoyl-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 (tricosanoic acid)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 4 routes

• Se-methylselenocysteineReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. S-Adenosyl-L-methionine:thiol S-methyltransferase

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

• prostaglandin H1Reach from here →
  1. De novo synthesis of 2Fe2S
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. prostaglandin G1 + AH2 = prostaglandin H1 + A + H2O

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

• (5S,11R)-dihydroxy-(6E,8Z,12E,14Z)-eicosatetraenoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (5S,11R)-dihydroxy-(6E,8Z,12E,14Z)-eicosatetraenoate + A + H2O

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

• (5S,15R)-dihydroxy-(6E,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. (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (5S,15R)-dihydroxy-(6E,8Z,11Z,13E)-eicosatetraenoate + A + H2O

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

• (5S,15S)-dihydroxy-(6E,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. (5S)-hydroxy-(6E,8Z,11Z,14Z)-eicosatetraenoate + AH2 + O2 = (5S,15S)-dihydroxy-(6E,8Z,11Z,13E)-eicosatetraenoate + A + H2O

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

• 17R-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate + AH2 + O2 = 17R-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoate + A + H2O

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

• 13-hydroxy-(4Z,7Z,10Z,14E,16Z,19Z)-docosahexaenoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate + AH2 + O2 = 13-hydroxy-(4Z,7Z,10Z,14E,16Z,19Z)-docosahexaenoate + A + H2O

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

• (18R)-hydroxy-(5Z,8Z,11Z,14Z,16E)-eicosapentaenoateReach 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,17Z)-eicosapentaenoate + AH2 + O2 = (18R)-hydroxy-(5Z,8Z,11Z,14Z,16E)-eicosapentaenoate + A + H2O

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

• (15R)-hydroxy-(5Z,8Z,11Z,13E,17Z)-eicosapentaenoateReach 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,17Z)-eicosapentaenoate + AH2 + O2 = (15R)-hydroxy-(5Z,8Z,11Z,13E,17Z)-eicosapentaenoate + A + H2O

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

• (11R)-hydroxy-(5Z,8Z,12E,14Z,17Z)-eicosapentaenoateReach 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,17Z)-eicosapentaenoate + AH2 + O2 = (11R)-hydroxy-(5Z,8Z,12E,14Z,17Z)-eicosapentaenoate + A + H2O

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

• 13R-hydroxy-(7Z,10Z,14E,16Z,19Z)-docosapentaenoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (7Z,10Z,13Z,16Z,19Z)-docosapentaenoate + AH2 + O2 = 13R-hydroxy-(7Z,10Z,14E,16Z,19Z)-docosapentaenoate + A + H2O

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

• (13S)-hydroxy-(9Z,11E)-octadecadienoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. (9Z,12Z)-octadecadienoate + AH2 + O2 = (13S)-hydroxy-(9Z,11E)-octadecadienoate + A + H2O

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 6 moremitochondrial matrix

• 2-(1-hydroxyethyl)thiamine diphosphateReach from here →
  1. De novo synthesis of 2Fe2S
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. pyruvate:thiamin diphosphate acetaldehydetransferase (decarboxylating)

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