L-cystine

Three steps

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

  Catalysed by SLC7A9, SLC3A1, KYNUextracellular space, cytosol

• D-alanyl-D-alanineReach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  2. alanine racemase
  3. Formation of Alanylalanine

  Catalysed by SLC7A9, SLC3A1extracellular space, cytosol

• Met-tRNA(Met)Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. L-Methionine:tRNAMet ligase (AMP-forming)

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

• (R)-glycerateReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. L-Serine:pyruvate aminotransferase
  3. D-glycerate:NADP+ 2-oxidoreductase

  Catalysed by CTH, AGXT, GRHPRcytosolOne of 4 routes

• CHEBI:16668Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. L-Cysteine:oxygen oxidoreductase
  3. 3-sulfino-L-alanine carboxy-lyase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, CDO1 and 2 morecytosol

• quinolinic acidReach from here →
  1. L-cystine(out) + L-aspartate(in) = L-cystine(in) + L-aspartate(out)
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. Transport of Quinolinic Acid

  Catalysed by SLC7A13, SLC25A14, SLC25A30, SLC22A6inner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• 3'-deoxy-3',4'-didehydro-CTPReach 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. CTP + AH2 + S-adenosyl-L-methionine = 3'-deoxy-3',4'-didehydro-CTP + 5'-deoxyadenosine + L-methionine + A + H2O + H(+)

  Catalysed by SLC7A9, SLC3A1, MOCOS, RSAD2extracellular space, cytosol

• cyanamideReach from here →
  1. L-cystine(out) + L-arginine(in) = L-cystine(in) + L-arginine(out)
  2. L-Arginine amidinohydrolase
  3. urea = cyanamide + H2O

  Catalysed by SLC7A9, ARG1, CA1, CA2inner side of the membrane, outer side of the membrane, cytosol

• pyridoxineReach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine-5'-phosphate phosphohydrolase

  Catalysed by SLC7A9, SLC3A1, SCLY, PHOSPHO2 and 1 moreextracellular space, cytosolOne of 2 routes

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

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

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

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

• tryptamineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-tryptophan decarboxy-lyase

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

• (S)-2-hydroxyglutarateReach 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. (S)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2

  Catalysed by SLC7A9, SLC3A1, MOCOS, L2HGDHextracellular space, cytosol

• formaldehydeReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. N(6),N(6)-dimethyl-L-lysyl(4)-[histone H3] + 2 A + 2 H2O = L-lysyl(4)-[histone H3] + 2 formaldehyde + 2 AH2

  Catalysed by TXNRD1, TXNRD3, TXNRD2, MOCOS and 2 morecytosolOne of 3 routes

• pantetheine 4'-phosphateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. (R)-4'-phosphopantothenate:L-cysteine ligase
  3. N-[(R)-4'-Phosphopantothenoyl]-L-cysteine carboxy-lyase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, PPCS and 1 morecytosolOne of 2 routes

• glyoxylic acidReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. glycine:oxygen oxidoreductase (deaminating)

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

• trans-1,2-dithiane-4,5-diolReach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. selenocysteine lyase (dithiothreitol)

  Catalysed by SLC7A9, SLC3A1, SCLYextracellular space, cytosol

• creatineReach from here →
  1. L-cystine(out) + L-arginine(in) = L-cystine(in) + L-arginine(out)
  2. L-Arginine:glycine amidinotransferase
  3. S-Adenosyl-L-methionine:guanidinoacetate N-methyltransferase

  Catalysed by SLC7A9, GATM, GAMTinner side of the membrane, outer side of the membrane, cytosolOne of 2 routes

• ferrocytochrome cReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. (S)-Lactate:NAD+ oxidoreductase
  3. (S)-Lactate:ferricytochrome-c 2-oxidoreductase

  Catalysed by CTH, LDHB, LDHAL6B, CYB5D1cytosol, mitochondrial matrixOne of 2 routes

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

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

• oxalic acidReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. transport of chloride and oxalate (cytosol to extracellular)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC6A14 and 1 morecytosol, extracellular spaceOne of 2 routes

• glycolaldehydeReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. L-Serine:pyruvate aminotransferase
  3. hydroxypyruvate carboxy-lyase (glycolaldehyde-forming)

  Catalysed by CTH, AGXTcytosolOne of 2 routes

• aldehydo-N-acetyl-D-mannosamineReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by CTH, NPLcytosolOne of 2 routes

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

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

• CHEBI:17208Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion
  3. glutathione:dehydroascorbate oxidoreductase

  Catalysed by TXNRD1, TXNRD3, TXNRD2cytosol

• homocysteineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion
  3. Formation of Homocystine

  Catalysed by TXNRD1, TXNRD3, TXNRD2cytosol

• L-glutamic 5-semialdehydeReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. L-Ornithine:2-oxo-acid aminotransferase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A6 and 1 morecytosol, extracellular space, mitochondrial matrix

• 17alpha-hydroxyprogesteroneReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. cytochrome P450 (17alpha-hydroxyprogesterone)

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

• anilineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Acetyl-CoA:arylamine N-acetyltransferase

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

• CHEBI:17312Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGScytosol, mitochondrial matrixOne of 2 routes

• all-trans-retinolReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  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 TXNRD1, TXNRD3, TXNRD2, MOCOS and 1 morecytosol

• sulfiteReach from here →
  1. cystine to cysteine conversion
  2. 2 glutathione + NADP(+) = glutathione disulfide + NADPH + H(+)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by GSR, TSTD1cytosolOne of 10 routes

• D-aspartic acidReach from here →
  1. L-cystine(out) + L-aspartate(in) = L-cystine(in) + L-aspartate(out)
  2. transport of aspartate (cytosol to extracellular)
  3. transport of D-aspartate (cytosol to extracellular)

  Catalysed by SLC7A13, SLC1A7, SLC1A3, SLC1A2 and 2 moreinner side of the membrane, outer side of the membrane, extracellular space, cytosol

• CHEBI:17380Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A5cytosol, extracellular spaceOne of 3 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A5 and 1 morecytosol, extracellular spaceOne of 3 routes

• enol-oxaloacetateReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by CTH, ME2, SDHB, SDHAcytosol

• S-methyl-5'-thioadenosineReach from here →
  1. L-cystine(out) + L-arginine(in) = L-cystine(in) + L-arginine(out)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase

  Catalysed by SLC7A9, SLC3A2, SLC7A7, SRMinner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• 5-aminolevulinic acidReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC3A2 and 5 morecytosol, extracellular space, mitochondrial matrix

• nitrateReach from here →
  1. L-cystine(out) + L-arginine(in) = L-cystine(in) + L-arginine(out)
  2. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)
  3. Fe(II)-heme b-[protein] + nitric oxide + O2 = Fe(III)-heme b-[protein] + nitrate

  Catalysed by SLC7A9, NOS3, NOS1, NOS2 and 1 moreinner side of the membrane, outer side of the membrane, cytosol

• indole-3-pyruvateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

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

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

  Catalysed by SLC7A9, SLC3A1, MOCOS, DIO1extracellular space, cytosol

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

  Catalysed by SLC7A9, SLC3A1, MOCOS, DIO1 and 1 moreextracellular space, cytosolOne of 2 routes

• 3,3'-diiodo-L-thyronine sulfateReach 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'-diiodo-L-thyronine sulfate + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine sulfate + AH2

  Catalysed by SLC7A9, SLC3A1, MOCOS, DIO1extracellular space, cytosolOne of 4 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

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

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

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC6A14 and 1 morecytosol, extracellular spaceOne of 3 routes

• 4-methylumbelliferyl acetateReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. 4-methylumbelliferyl acetate + H2O = 4-methylumbelliferone + acetate + H(+)

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

• CHEBI:17769Reach from here →
  1. L-cystine(out) + L-arginine(in) = L-cystine(in) + L-arginine(out)
  2. Putrescine Export via Putrescine/Arginine Antiporter
  3. Putrescine:oxygen oxidoreductase (deaminating)

  Catalysed by SLC7A9, SLC3A2, SLC7A7, AOC2 and 2 moreinner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• trans-urocanateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and histidine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A6 and 1 morecytosol, extracellular spaceOne of 2 routes

• urateReach from here →
  1. cystine to cysteine conversion
  2. 2 glutathione + NADP(+) = glutathione disulfide + NADPH + H(+)
  3. urate(out) + glutathione(in) = urate(in) + glutathione(out)

  Catalysed by GSR, SLC22A13cytosol, inner side of the membrane, outer side of the membraneOne of 6 routes

• 5-hydroxy-L-tryptophanReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin

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