iron(2+)

Three steps

• S-sulfanyl-L-cysteineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. 2 L-cysteine = S-sulfanyl-L-cysteine + L-alanine

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

• N-formyl-L-kynurenineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 7 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• (3S)-citramalyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

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

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

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

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

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

• 5-phospho-beta-D-ribosylamineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and glutamine (cytosol to extracellular)
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 7 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• D-glucosamine 6-phosphateReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and glutamine (cytosol to extracellular)
  3. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

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

• L-methionine (S)-S-oxideReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

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

• L-methionine (R)-S-oxideReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 7 moremitochondrial matrix, extracellular space, cytosol

• 5-amino-2-oxopentanoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

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

• deoxycholoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, cytosolOne of 4 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-Alanine:glyoxylate aminotransferase
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

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

• methylamineReach from here →
  1. De novo synthesis of 2Fe2S
  2. N(omega)-methyl-L-arginine + pyruvate = 5-(3-methylguanidino)-2-oxopentanoate + L-alanine
  3. N(omega)-methyl-L-arginine + H2O = L-citrulline + methylamine

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

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosolOne of 4 routes

• pyridoxal 5'-phosphateReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate + O2 = pyridoxal 5'-phosphate + H2O2

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

• an L-alpha-amino acidReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. an L-alpha-amino acid(in) + L-kynurenine(out) = an L-alpha-amino acid(out) + L-kynurenine(in)

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

• 4-aminobutanoateReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and chloride (cytosol to extracellular)
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 5 moremitochondrial matrix, extracellular space, cytosolOne of 4 routes

• 3-iodo-L-tyrosineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

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

• N-benzoylglycineReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-Alanine:glyoxylate aminotransferase
  3. Hippurate (Benzoylglycinate) Formation

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

• 3-sulfino-L-alanineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. L-Cysteine:oxygen oxidoreductase

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

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

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 9 moremitochondrial matrix, cytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Carnitine O-Palmitoyltransferase

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

• (9Z)-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 (palmitolate)

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

• Fe(II)-heme aReach from here →
  1. Ferrochelatase, Mitochondrial
  2. farnesyl-PP[m] + H2O[m] + heme[m] = PPi[m] + Fe(II)-heme o[m]
  3. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2

  Catalysed by FECH, COX10, COX15mitochondrial matrix

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. De novo synthesis of 2Fe2S
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

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

• 3-hydroxyisovaleryl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

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

• (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylateReach from here →
  1. De novo synthesis of 2Fe2S
  2. Transport of Hydroxy-Proline
  3. trans-4-Hydroxy-L-proline:NAD+ 5-oxidoreductase

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 7 moremitochondrial matrix, extracellular space, cytosolOne of 5 routes

• CHEBI:63544Reach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

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

• hexacosanoyl-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 (cerotic acid)

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

• tetracosanoyl-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 (lignocerate)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 10 routes

• docosanoyl-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 (behenic acid)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 9 routes

• a 2,3-saturated acyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. a 2,3-saturated acyl-CoA + A = a 2,3-dehydroacyl-CoA + AH2

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

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. De novo synthesis of 2Fe2S
  2. transport of alanine and arginine (cytosol to extracellular)
  3. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 7 moremitochondrial matrix, extracellular space, cytosol

• xanthurenateReach from here →
  1. De novo synthesis of 2Fe2S
  2. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)
  3. 3-hydroxy-L-kynurenine + glyoxylate = xanthurenate + glycine + H2O

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

• (2E)-octadecenoyl-CoAReach from here →
  1. De novo synthesis of 2Fe2S
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Carnitine O-Palmitoyltransferase

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

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-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 (stearidonic acid)

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

• (4S)-4-hydroxy-2-oxoglutarateReach from here →
  1. De novo synthesis of 2Fe2S
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (4S)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

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

• L-threonylcarbamoyladenylateReach from here →
  1. De novo synthesis of 2Fe2S
  2. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)
  3. L-threonine + hydrogencarbonate + ATP = L-threonylcarbamoyladenylate + diphosphate + H2O

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

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-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 (EPA)

  Catalysed by NDUFAB1, FDXR, FXN, FDX2 and 11 moremitochondrial matrix, cytosolOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-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 (DPA)

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

• (9Z,12Z,15Z)-octadecatrienoyl-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 (linolenate)

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

• (13Z)-docosenoyl-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 (cis-erucic acid)

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

• (11Z)-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 (cis-gondoic acid)

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

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-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 ((9Z,12Z,15Z,18Z,21Z)-TPA)

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

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-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,12Z,15Z,18Z,21Z)-THA)

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

• (15Z)-tetracosenoyl-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 (nervonic acid)

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

• (17Z)-hexacosenoyl-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 (ximenic acid)

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

• (8Z,11Z,14Z)-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 (dihomo-gamma-linolenate)

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

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-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 (omega-3-arachidonic acid)

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

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-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 (DHA)

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