L-cystine

Three steps

• N-formyl-L-kynurenineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

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

• (S)-3-amino-2-methylpropanoateReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-3-amino-isobutanoate:2-oxoglutarate aminotransferase

  Catalysed by CTH, AGXT2, ABATcytosol, mitochondrial matrix

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

  Catalysed by SLC7A9, SLC3A1, KYNU, AADATextracellular space, cytosol

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

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

• 5-phospho-beta-D-ribosylamineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

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

• phosphoenolpyruvateReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. Pyruvate:carbon-dioxide ligase (ADP-forming)
  3. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2

  Catalysed by CTH, PC, PCK1, PCK2cytosol, mitochondrial matrix

• an N-acetyl-L-cysteine-S-conjugateReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. an N-acetyl-L-cysteine-S-conjugate + H2O = an S-substituted L-cysteine + acetate

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

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

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

• L-methionine (S)-S-oxideReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

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

• L-methionine (R)-S-oxideReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC7A5 and 3 morecytosol, extracellular space

• deoxycholoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 2 morecytosol, mitochondrial matrixOne of 4 routes

• S-sulfanylglutathioneReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. Sulfide:glutathione,quinone oxidoreductase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, CTH and 1 morecytosol, mitochondrial matrixOne of 9 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

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

• methylamineReach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  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 SLC7A9, SLC3A1, AGXT2, DDAH1extracellular space, cytosol

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. L-cystine(out) + L-aspartate(in) = L-cystine(in) + L-aspartate(out)
  2. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine

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

• pyridoxal 5'-phosphateReach 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 + O2 = pyridoxal 5'-phosphate + H2O2

  Catalysed by SLC7A9, SLC3A1, SCLY, PNPOextracellular space, cytosol

• 2-hydroxy-4-methylvaleric 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 reduction

  Catalysed by SLC7A9, SLC3A1, BCAT2, SDR42E2 and 17 moreextracellular space, cytosol, mitochondrial matrix

• an L-alpha-amino acidReach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  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 SLC7A9, SLC3A1, KYNU, SLC7A11extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 4-aminobutanoateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC6A14 and 1 morecytosol, extracellular spaceOne of 5 routes

• 3-iodo-L-tyrosineReach 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 2 routes

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

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

• (2S)-2-phospholactateReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (2S)-2-phospholactate + H2O = (S)-lactate + phosphate

  Catalysed by CTH, LDHA, LDHB, LDHC and 3 morecytosolOne of 2 routes

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

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

• a 2,3-dehydroacyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  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 TXNRD1, TXNRD3, TXNRD2, MOCOS and 1 morecytosol

• estrone 3-sulfateReach 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. estrone 3-sulfate + H2O = estrone + sulfate + H(+)

  Catalysed by SLC7A13, SLC25A14, SLC25A30, STSinner side of the membrane, outer side of the membrane

• prostaglandin E2Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin E2 (cytosol to extracellular)

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

• N-benzoylglycineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. Hippurate (Benzoylglycinate) Formation

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC3A2 and 2 morecytosol, extracellular space, mitochondrial matrixOne of 2 routes

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

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 6 morecytosol, mitochondrial matrixOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Carnitine O-Palmitoyltransferase

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

• (9Z)-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 (palmitolate)

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

• Fe(II)-heme aReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2

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

• (R)-4'-phosphopantetheineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. (R)-4'-phosphopantothenate:L-cysteine ligase
  3. N-[(R)-4-phosphopantothenoyl]-L-cysteine + H(+) = (R)-4'-phosphopantetheine + CO2

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

• 3-hydroxyisovaleryl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

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

• (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylateReach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  2. Transport of Hydroxy-Proline
  3. trans-4-Hydroxy-L-proline:NAD+ 5-oxidoreductase

  Catalysed by SLC7A9, SLC3A1, SLC1A4, PYCR1 and 2 moreextracellular space, cytosolOne of 5 routes

• (5-hydroxyindol-3-yl)acetateReach 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 5-Hydroxyindoleacetate

  Catalysed by SLC7A13, SLC25A14, SLC25A30, SLC4A1inner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• dTMPReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. pyruvate kinase (dTDP)
  3. DTDP Diphosphohydrolase

  Catalysed by CTH, PKM, PKLR, ENTPD3 and 2 morecytosol, extracellular spaceOne of 5 routes

• CHEBI:63544Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

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

• ubiquinol-10Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. ubiquinone-10 + hydrogen sulfide + sulfite + 2 H(+) = ubiquinol-10 + thiosulfate

  Catalysed by TXNRD1, TXNRD3, TXNRD2, CTH and 1 morecytosolOne of 3 routes

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

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

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

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 8 morecytosol, mitochondrial matrixOne of 10 routes

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

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

• a 2,3-saturated acyl-CoAReach 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. a 2,3-saturated acyl-CoA + A = a 2,3-dehydroacyl-CoA + AH2

  Catalysed by SLC7A9, SLC3A1, MOCOS, ACAD10extracellular space, cytosol

• xanthurenateReach 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 + glyoxylate = xanthurenate + glycine + H2O

  Catalysed by SLC7A9, SLC3A1, KYNU, KYAT3 and 1 moreextracellular space, cytosol

• (2E)-octadecenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Carnitine O-Palmitoyltransferase

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

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

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

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

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

• L-threonylcarbamoyladenylateReach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  2. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)
  3. L-threonine + hydrogencarbonate + ATP = L-threonylcarbamoyladenylate + diphosphate + H2O

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

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (EPA)

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

• (7Z,10Z,13Z,16Z,19Z)-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 (DPA)

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

• (9Z,12Z,15Z)-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 (linolenate)

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