L-cystine

Three steps

• D-lysineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. D-lysine(in) + L-ornithine(out) = D-lysine(out) + L-ornithine(in)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A6 and 1 morecytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-arginineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. D-arginine(in) + L-ornithine(out) = D-arginine(out) + L-ornithine(in)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A6 and 1 morecytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• cis-aconitic acidReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. pyruvate kinase (dTDP)
  3. transport of cis-aconitate and PEP (cytosol to mitochondria)

  Catalysed by CTH, PKM, PKLR, SLC25A1cytosol, mitochondrial matrixOne of 5 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A5 and 1 morecytosol, extracellular space

• tetra-mu3-sulfido-tetrairon(2+)Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. De novo synthesis of 2Fe2S
  3. Synthesis of 4Fe4S from 2Fe2S

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NDUFAB1 and 12 morecytosol, mitochondrial matrix

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

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLCO1A2 and 1 morecytosol, extracellular spaceOne of 4 routes

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and isoleucine (cytosol to extracellular)
  3. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A5 and 2 morecytosol, extracellular space

• 2-oxohexanoateReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by CTH, AGXT2cytosol

• L-alpha-aminobutyric acidReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. glutamic-pyruvic transaminase ((S)-2-aminobutanoate)

  Catalysed by CTH, AGXT2, GPTcytosol

• 5-aminolevulinateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

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

• 3-methyl-2-oxovaleric acidReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and isoleucine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A5 and 1 morecytosol, extracellular spaceOne of 2 routes

• 3-(4-hydroxyphenyl)pyruvateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. L-tyrosine:2-oxoglutarate aminotransferase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A5 and 3 morecytosol, extracellular spaceOne of 6 routes

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

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

• taurocholateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion
  3. solute carrier organic anion transporter (GSH)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLCO1A2 and 1 morecytosol, extracellular spaceOne of 3 routes

• glycochenodeoxycholic acidReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. bile acid-CoA: amino acid N-acyltransferase

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

• 3-hydroxyanthranilateReach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  2. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by SLC7A9, SLC3A1, KYNUextracellular space, cytosol

• glyoxylateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. glycine + 2-oxoglutarate = glyoxylate + L-glutamate

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC3A2 and 3 morecytosol, extracellular spaceOne of 20 routes

• 3-hydroxyisovaleric acidReach from here →
  1. Transport of L-Cystine (L-Leucine antiport) by B0, +At Transporter
  2. L-Leucine:2-oxoglutarate aminotransferase
  3. 4-methyl-2-oxopentanoate decarboxylation

  Catalysed by SLC7A9, SLC3A1, BCAT2, ALOX12B and 1 moreextracellular space, cytosol, mitochondrial matrix

• glycolithocholic acidReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. bile acid-CoA:amino acid N-acyltransferase (glycine)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC3A2 and 3 morecytosol, extracellular spaceOne of 4 routes

• L-2-amino-3-oxobutanoic acidReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. Acetyl-CoA:glycine C-acetyltransferase

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

• 25-hydroxycholesterolReach 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. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O

  Catalysed by SLC7A9, SLC3A1, MOCOS, CH25Hextracellular space, cytosol

• (3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA

  Catalysed by CTH, HMGCL, HMGCLL1cytosol, mitochondrial matrix

• 13-cis-retinalReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. transport of pyruvate and retinoate (cytosol to extracellular)
  3. retinal dehydrogenase (13-cis-retinal)

  Catalysed by CTH, SLC16A4, SLC16A5, SLC16A3 and 5 morecytosol, extracellular space, endoplasmic reticulum

• tert-butanolReach from here →
  1. cystine to cysteine conversion
  2. 2 glutathione + NADP(+) = glutathione disulfide + NADPH + H(+)
  3. tert-butyl hydroperoxide + 2 glutathione = tert-butanol + glutathione disulfide + H2O

  Catalysed by GSR, CLIC2, GPX1, GPX2 and 2 morecytosol

• N-(acetyl)-sphing-4-enineReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate

  Catalysed by CTH, SLC16A4, SLC16A5, SLC16A3 and 3 morecytosol, extracellular space

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

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

• taurineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 1 morecytosol, mitochondrial matrixOne of 5 routes

• all-trans-13,14-dihydroretinolReach 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. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by SLC7A9, SLC3A1, MOCOS, RETSATextracellular space, cytosol

• 3,3',5-triiodo-L-thyronineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion
  3. transport of GSH and triiodothyronine (cytosol to extracellular)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLCO1A2 and 1 morecytosol, extracellular spaceOne of 3 routes

• 3,3',5'-triiodo-L-thyronineReach 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. 3,3',5'-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2

  Catalysed by SLC7A9, SLC3A1, MOCOS, DIO1 and 1 moreextracellular space, cytosol

• acetoacetyl-CoAReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. acetoacetate + ATP + CoA = acetoacetyl-CoA + AMP + diphosphate

  Catalysed by CTH, AACScytosol, mitochondrial matrixOne of 2 routes

• (S)-malyl-CoAReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate

  Catalysed by CTH, ME2, SUCLG1, SUCLA2cytosol

• (R)-methylmalonyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 1 morecytosol, mitochondrial matrixOne of 3 routes

• (S)-methylmalonyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGScytosol, mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. acid phosphatase 2, lysosomal (CoA)

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

• (2E)-dodecenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGScytosol, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

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

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

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

• butanoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 6 morecytosol, mitochondrial matrixOne of 11 routes

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

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 9 routes

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

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

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

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 12 routes

• itaconyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Itaconate:CoA ligase (GDP-forming)

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

• (9Z,12Z)-octadecadienoyl-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)-octadecadienoic acid)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 13 routes

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

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 11 routes

• octanoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 1 morecytosol, mitochondrial matrixOne of 20 routes

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

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGScytosol, mitochondrial matrixOne of 5 routes

• propanoyl-CoAReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine
  3. 2-methyl-3-oxopropanoate + NAD(+) + CoA + H2O = propanoyl-CoA + hydrogencarbonate + NADH + H(+)

  Catalysed by CTH, AGXT2, ALDH6A1cytosol

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

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 7 morecytosol, mitochondrial matrixOne of 15 routes

• prostaglandin E1Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin E1 (cytosol to extracellular)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLCO2A1cytosol, extracellular spaceOne of 4 routes