L-cystine

Three steps

• a quinolReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. a quinone + hydrogen sulfide + glutathione + H(+) = S-sulfanylglutathione + a quinol

  Catalysed by TXNRD1, TXNRD3, TXNRD2, CTH and 1 morecytosolOne of 5 routes

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

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC6A14 and 1 morecytosol, extracellular spaceOne of 4 routes

• folic 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. Folate Transport by Oats

  Catalysed by SLC7A13, SLC25A14, SLC25A30, SLC22A8 and 1 moreinner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• phenylacetylglycineReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

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

• CHEBI:27540Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. medium-chain acyl-CoA ligase (hexanoic acid)

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

• selenomethionineReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. Selenomethionine methanethiol-lyase (deaminating)

  Catalysed by CTH, AGXT2cytosol

• CHEBI:27760Reach from here →
  1. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. Selenocystathionine Lysteine-lyase (deaminating)

  Catalysed by SLC7A9, SLC3A1, SCLY, CTHextracellular space, cytosolOne of 2 routes

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

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

• 5-hydroxytryptophanReach 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 2 routes

• 5(S)-HETEReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion
  3. Glutathione: 5-HPETE oxidoreductase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, GPX5 and 8 morecytosolOne of 2 routes

• 3-mercaptolactic acidReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-Mercaptolactate:NAD+ oxidoreductase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, GOT2 and 5 morecytosol

• 2-oxopentanoateReach 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-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by CTH, AGXT2cytosol

• CHEBI:28666Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. LTD4 hydrolysis

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

• CHEBI:28701Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by TXNRD1, TXNRD3, TXNRD2, NAGS and 3 morecytosol, mitochondrial matrix, peroxisomeOne of 7 routes

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

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

• a dicarboxylateReach 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. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)

  Catalysed by SLC7A13, SLC25A14, SLC25A30, SLC25A35 and 1 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• N-glycoloylneuraminateReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. CMP-N-acetylneuraminate monooxygenase (N-acetylneuraminate)

  Catalysed by CTH, NPLcytosol

• a carboxylateReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by CTH, FAHD1cytosol

• L-ascorbic acidReach from here →
  1. cystine to cysteine conversion
  2. 2 glutathione + NADP(+) = glutathione disulfide + NADPH + H(+)
  3. L-dehydroascorbate + 2 glutathione = glutathione disulfide + L-ascorbate

  Catalysed by GSR, CLIC1, GSTO1, GSTO2cytosol

• Ca(2+)Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)
  3. Ca(2+)(out) + K(+)(out) + 4 Na(+)(in) = Ca(2+)(in) + K(+)(in) + 4 Na(+)(out)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC1A1 and 5 morecytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• 3-methyl-1-hydroxybutylthiamine diphosphateReach from here →
  1. Transport of L-Cystine (L-Leucine antiport) by B0, +At Transporter
  2. L-Leucine:2-oxoglutarate aminotransferase
  3. branched chain keto acid dehydrogenase E1 (4-methyl-2-oxopentanoate)

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

• Phe-tRNA(Phe)Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-Phenylalanine:tRNA(Ala) ligase (AMP-forming)

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

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

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

• Trp-tRNA(Trp)Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-Tryptophan -tRNA(Trp) ligase (AMP-forming)

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

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

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

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

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

• isocyanateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. L-citrulline + H(+) = isocyanate + L-ornithine

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

• a thiolReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. L-cysteinyl-[sulfatase] + 2 a thiol + O2 = an organic disulfide + 3-oxo-L-alanyl-[sulfatase] + hydrogen sulfide + H2O + H(+)

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

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

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

• hydrogenselenideReach 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. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)

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

• glycocholateReach 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 6 routes

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

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

• D-aspartateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)
  3. D-aspartate(out) + K(+)(in) + 3 Na(+)(out) + H(+)(out) = D-aspartate(in) + K(+)(out) + 3 Na(+)(in) + H(+)(in)

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC1A1 and 4 morecytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• CHEBI:30249Reach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)

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

• 3-acetamidopropanalReach from here →
  1. L-cystine(out) + L-arginine(in) = L-cystine(in) + L-arginine(out)
  2. Transport by Y+Lat-2 into Extracellular Space
  3. N1-acetylspermidine:oxygen oxidoreductase (3-acetamidopropanal-forming)

  Catalysed by SLC7A9, SLC3A2, SLC7A6, PAOX and 1 moreinner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• oxalateReach 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. oxalate(in) + sulfate(out) = oxalate(out) + sulfate(in)

  Catalysed by SLC7A13, SLC25A14, SLC25A30, SLC26A2 and 2 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• anthranilic acidReach 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 hydrolase

  Catalysed by SLC7A9, SLC3A1, KYNUextracellular space, cytosol

• citric acidReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. Pyruvate:carbon-dioxide ligase (ADP-forming)
  3. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)

  Catalysed by CTH, PC, CScytosol, mitochondrial matrixOne of 9 routes

• maleateReach 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. maleate(in) + sulfate(out) = maleate(out) + sulfate(in)

  Catalysed by SLC7A13, SLC25A14, SLC25A30inner side of the membrane, outer side of the membraneOne of 2 routes

• malonic acidReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. Malonate Transport, Antiport with Chloride

  Catalysed by TXNRD1, TXNRD3, TXNRD2, SLC6A14cytosol, extracellular spaceOne of 4 routes

• (9Z)-octadecenoateReach from here →
  1. Transport of L-Cystine (L-Leucine antiport) by B0, +At Transporter
  2. N-(9Z-octadecenoyl)-L-leucine + H2O = L-leucine + (9Z)-octadecenoate
  3. N-(9Z-octadecenoyl)-L-leucine + H2O = L-leucine + (9Z)-octadecenoate

  Catalysed by SLC7A9, SLC3A1, PM20D1extracellular space, cytosol

• (S)-dihydroorotateReach from here →
  1. L-cystine(out) + L-aspartate(in) = L-cystine(in) + L-aspartate(out)
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  3. (S)-dihydroorotate amidohydrolase

  Catalysed by SLC7A13, CADinner side of the membrane, outer side of the membrane, cytosolOne of 2 routes

• an alcoholReach from here →
  1. cystine to cysteine conversion
  2. 2 glutathione + NADP(+) = glutathione disulfide + NADPH + H(+)
  3. a hydroperoxide + 2 glutathione = an alcohol + glutathione disulfide + H2O

  Catalysed by GSR, CP, PRDX6cytosol

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

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

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. L-cystine thiocysteine-lyase (deaminating
  2. Pyruvate:carbon-dioxide ligase (ADP-forming)
  3. D-aspartate:2-oxoglutarate aminotransferase

  Catalysed by CTH, PC, GOT2cytosol, mitochondrial matrix

• (2R,3R)-tartrateReach 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. (2R,3R)-tartrate(in) + sulfate(out) = (2R,3R)-tartrate(out) + sulfate(in)

  Catalysed by SLC7A13, SLC25A14, SLC25A30inner side of the membrane, outer side of the membraneOne of 2 routes

• (S,S)-tartrateReach 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. (S,S)-tartrate(in) + sulfate(out) = (S,S)-tartrate(out) + sulfate(in)

  Catalysed by SLC7A13, SLC25A14, SLC25A30inner side of the membrane, outer side of the membraneOne of 2 routes

• (3R)-citramalateReach 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. (3R)-citramalate(in) + sulfate(out) = (3R)-citramalate(out) + sulfate(in)

  Catalysed by SLC7A13, SLC25A14, SLC25A30inner side of the membrane, outer side of the membraneOne of 2 routes

• (3S)-citramalateReach 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. (3S)-citramalate(in) + sulfate(out) = (3S)-citramalate(out) + sulfate(in)

  Catalysed by SLC7A13, SLC25A14, SLC25A30inner side of the membrane, outer side of the membraneOne of 2 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion
  3. transport of arachidonate and GSH (cytosol to extracellular)

  Catalysed by TXNRD1, TXNRD3, TXNRD2cytosol, extracellular spaceOne of 2 routes