L-cystine

Three steps

• (13Z)-docosenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 7 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (DHA)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 7 routes

• heptadecanoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 5 routes

• nonadecanoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 3 morecytosol, mitochondrial matrixOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Activation of dodecanedioic acid

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGScytosol, mitochondrial matrixOne of 4 routes

• decanedioyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Production of Sebacoylcarnitine

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 3 morecytosol, mitochondrial matrixOne of 4 routes

• octanedioyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Production of Suberylcarnitine

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 3 morecytosol, mitochondrial matrixOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGScytosol, mitochondrial matrixOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 5 routes

• 2-iminobutanoateReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. 2-iminobutanoate + H2O = 2-oxobutanoate + NH4(+)

  Catalysed by CTH, AGXT2, RIDAcytosol

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 3 morecytosol, mitochondrial matrix

• (13Z)-eicosenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 5 routes

• 2-aminoprop-2-enoateReach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  2. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)
  3. D-serine = 2-aminoprop-2-enoate + H2O

  Catalysed by SLC7A9, SLC3A1, SLC7A10, SRRextracellular space, cytosol, inner side of the membrane, outer side of the membrane

• 20-hydroxy-(5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion
  3. Resistance-Nodulation-Cell Division (Rnd) Tcdb:2.A.60.1.2

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• N-acetyl-L-aspartyl-L-glutamateReach from here →
  1. L-cystine(out) + L-aspartate(in) = L-cystine(in) + L-aspartate(out)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. N-acetyl-L-aspartate + L-glutamate + ATP = N-acetyl-L-aspartyl-L-glutamate + ADP + phosphate + H(+)

  Catalysed by SLC7A13, NAT8L, RIMKLA, RIMKLBinner side of the membrane, outer side of the membrane, mitochondrial matrix

• N-acetyl-L-aspartyl-L-glutamyl-L-glutamateReach from here →
  1. L-cystine(out) + L-aspartate(in) = L-cystine(in) + L-aspartate(out)
  2. acetyl-CoA:L-aspartate N-acetyltransferase
  3. N-acetyl-L-aspartate + 2 L-glutamate + 2 ATP = N-acetyl-L-aspartyl-L-glutamyl-L-glutamate + 2 ADP + 2 phosphate + 2 H(+)

  Catalysed by SLC7A13, NAT8L, RIMKLAinner side of the membrane, outer side of the membrane, mitochondrial matrix

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 2 morecytosol, mitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 4 morecytosol, mitochondrial matrix, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Production of Hexadecanedioylcarnitine

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 3 morecytosol, mitochondrial matrixOne of 2 routes

• pristanoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. fatty-acid-CoA ligase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 1 morecytosol, mitochondrial matrixOne of 2 routes

• undecanoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 6 morecytosol, mitochondrial matrixOne of 3 routes

• (9S)-hydroxy-(10E,12Z)-octadecadienoateReach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  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 SLC7A9, SLC3A1, MOCOS, PTGS1 and 1 moreextracellular space, cytosolOne of 2 routes

• (9R)-hydroxy-(10E,12Z)-octadecadienoateReach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  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 SLC7A9, SLC3A1, MOCOS, PTGS1 and 1 moreextracellular space, cytosol

• ursodeoxycholateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion
  3. transport of GSH and ursodeoxycholate (cytosol to extracellular)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLCO1A2 and 1 morecytosol, extracellular spaceOne of 2 routes

• (11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoateReach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  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 SLC7A9, SLC3A1, MOCOS, PTGS2extracellular space, cytosol

• (15R)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  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 SLC7A9, SLC3A1, MOCOS, PTGS2extracellular space, cytosol

• prostaglandin G2Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin G2 (cytosol to extracellular)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• glycodeoxycholateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. Glycodeoxycholate amidohydrolase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC3A2 and 2 morecytosol, extracellular spaceOne of 3 routes

• (12R)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoateReach from here →
  1. cystine to cysteine conversion
  2. 2 glutathione + NADP(+) = glutathione disulfide + NADPH + H(+)
  3. (12R)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + 2 glutathione = (12R)-hydroxy-(5Z,8Z,10E,14Z)-eicosatetraenoate + glutathione disulfide + H2O

  Catalysed by GSR, GPX1, GPX2, GPX4cytosol

• (5Z)-tetradecenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (physeteric acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 8 routes

• (3E)-decenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 4 morecytosol, mitochondrial matrix

• 2-glyceryl-prostaglandin H2Reach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  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 SLC7A9, SLC3A1, MOCOS, PTGS2extracellular space, cytosol

• N-tetradecanoylglycineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. tetradecanoyl-CoA + glycine = N-tetradecanoylglycine + CoA + H(+)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC3A2 and 3 morecytosol, extracellular space

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 3 morecytosol, mitochondrial matrix

• N-eicosanoylglycinateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. eicosanoyl-CoA + glycine = N-eicosanoylglycinate + CoA + H(+)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC3A2 and 3 morecytosol, extracellular space

• N-docosanoylglycineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. docosanoyl-CoA + glycine = N-docosanoylglycine + CoA + H(+)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC3A2 and 3 morecytosol, extracellular space

• N-hexacosanoylglycineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. hexacosanoyl-CoA + glycine = N-hexacosanoylglycine + CoA + H(+)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC3A2 and 3 morecytosol, extracellular space

• an N(2)-acyl-L-glutamineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC3A2 and 4 morecytosol, extracellular space

• (7Z)-hexadecenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 8 routes