L-cystine

Three steps

• 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + L-glutamine + ATP + H2O = 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + L-glutamate + ADP + phosphate + H(+)

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

• kinetensin-(1-8)Reach from here →
  1. Transport of L-Cystine (L-Leucine antiport) by B0, +At Transporter
  2. Chymase
  3. kinetensin + H2O = kinetensin-(1-8) + L-leucine

  Catalysed by SLC7A9, SLC3A1, CMA1, ACE2extracellular space, cytosol

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

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

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

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

• (7Z)-octadecenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)

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

• acetaldehydeReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and threonine (cytosol to extracellular)
  3. L-threonine acetaldehyde-lyase (glycine-forming)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A5cytosol, extracellular spaceOne of 2 routes

• CHEBI:15345Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Acetoacetate:CoA ligase (AMP-forming)

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

• acetoneReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. Acetoacetate carboxy-lyase (acetone-forming)

  Catalysed by CTHcytosol, mitochondrial matrix

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

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

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

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

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A5 and 2 morecytosol, extracellular spaceOne of 3 routes

• CHEBI:15380Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 2 morecytosol, mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

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

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

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

• isobutyryl-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-methyl-pentanoyl-CoA)

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

• 3-methylbut-2-enoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 1 morecytosol, mitochondrial matrix

• isovaleryl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. glycine N-acyltransferase (isovalerylglycine)

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

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

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

• CHEBI:15519Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. propanoyl-CoA C-acyltransferase

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

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

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

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

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 3 morecytosol, mitochondrial matrixOne of 11 routes

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

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

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

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 3 morecytosol, mitochondrial matrixOne of 48 routes

• CHEBI:15611Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. S-Adenosyl-L-methionine:glycine N-methyltransferase

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

• 10-formyltetrahydrofolic acidReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. transport of formate and pyruvate (cytosol to extracellular)
  3. Formate:tetrahydrofolate ligase (ADP-forming)

  Catalysed by CTH, SLC16A4, SLC16A5, SLC16A7 and 3 morecytosol, extracellular spaceOne of 2 routes

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach 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 spaceOne of 3 routes

• CHEBI:15650Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 32 morecytosol, mitochondrial matrixOne of 4 routes

• betaine aldehydeReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. choline + A = betaine aldehyde + AH2

  Catalysed by TXNRD1, TXNRD3, TXNRD2, MOCOS and 1 morecytosol

• trimethylenediamineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and lysine (cytosol to extracellular)
  3. deoxyhypusine synthase (1,3-diaminopropane)

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

• CHEBI:15727Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)

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

• CHEBI:15746Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. diamine N-acetyltransferase (N1-acetylspermine)

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

• malonateReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. malonate(out) + (S)-malate(in) = malonate(in) + (S)-malate(out)

  Catalysed by CTH, ME2, SLC25A10cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• bromideReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion
  3. 2-bromoacetaldehyde:glutathione S-(formylmethyl)transferase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, MGST3 and 16 morecytosolOne of 2 routes

• alpha-D-glucosamine 6-phosphateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

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

• N-acetyl-beta-D-glucosaminylamineReach from here →
  1. L-cystine(out) + L-aspartate(in) = L-cystine(in) + L-aspartate(out)
  2. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by SLC7A13, AGAinner side of the membrane, outer side of the membrane

• thioredoxin dithiolReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. L-Cysteine:tRNA(Cys) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by TXNRD1, TXNRD3, TXNRD2, CARS1cytosolOne of 6 routes

• methanethiolReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. methanethiol + O2 + H2O = hydrogen sulfide + formaldehyde + H2O2 + H(+)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, CTH and 1 morecytosol

• a halide anionReach from here →
  1. cystine to cysteine conversion
  2. 2 glutathione + NADP(+) = glutathione disulfide + NADPH + H(+)
  3. RX + glutathione = an S-substituted glutathione + a halide anion + H(+)

  Catalysed by GSR, GSTT4, GSTA4, GSTZ1 and 20 morecytosol

• CHEBI:16174Reach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. pyruvate kinase (dTDP)
  3. nucleoside-diphosphate kinase (dADP)

  Catalysed by CTH, PKM, PKLR, NME4 and 5 morecytosolOne of 3 routes

• CHEBI:16284Reach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. pyruvate kinase (dTDP)
  3. deoxythymidylate kinase (dADP)

  Catalysed by CTH, PKM, PKLR, DTYMKcytosol, mitochondrial matrixOne of 3 routes

• nitriteReach 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. 4 nitric oxide + O2 + 2 H2O = 4 nitrite + 4 H(+)

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

• 11-cis-retinolReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 5 morecytosol, mitochondrial matrix

• cob(II)alaminReach 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 cob(II)alamin + A + 2 H2O + 2 H(+) = 2 aquacob(III)alamin + AH2

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

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

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

• iodideReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A5 and 1 morecytosol, extracellular spaceOne of 25 routes

• CHEBI:16497Reach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. pyruvate kinase (dTDP)
  3. dGTP:pyruvate 2-O-phosphotransferase

  Catalysed by CTH, PKM, PKLRcytosol

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

  Catalysed by SLC7A9, SLC3A1, SCLYextracellular space, cytosolOne of 3 routes

• CHEBI:16523Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. transport of D-serine and glycine (cytosol to extracellular)

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

• N(2)-acetyl-L-ornithineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. N2-Acetyl-L-ornithine amidohydrolase

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

• 2-phenylacetamideReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

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