L-cysteine

Two steps

• 6-amino-2-oxohexanoateReach from here →
  1. transport of cysteine and lysine (cytosol to extracellular)
  2. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by SLC7A6extracellular space, cytosol, peroxisome

• L-homocysteineReach from here →
  1. transport of cysteine and methionine (cytosol to extracellular)
  2. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by SLC7A5, BHMT2extracellular space, cytosol

• carbamoyl phosphateReach from here →
  1. transport of cysteine and glutamine (cytosol to extracellular)
  2. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)

  Catalysed by SLC3A2, SLC7A5, SLC7A10, CADextracellular space, cytosolOne of 4 routes

• S-methyl-L-methionineReach from here →
  1. transport of cysteine and methionine (cytosol to extracellular)
  2. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by SLC7A5, BHMT2extracellular space, cytosol

• glutathione disulfideReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion

  Catalysed by TXNRD1, TXNRD3, TXNRD2cytosolOne of 14 routes

• taurolithocholate 3-sulfateReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and sulfotaurolithocholate (cytosol to extracellular)

  Catalysed by SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• L-tyrosineReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by SLC3A2, SLC7A5, SLC7A8, AADATextracellular space, cytosolOne of 2 routes

• N(omega),N(omega)-dimethyl-L-arginineReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by SLC3A2, SLC7A5, SLC7A8, AGXT2extracellular space, cytosolOne of 6 routes

• an acyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 1 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• trans-4-hydroxy-L-prolineReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by SLC7A5, SLC1A4extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• histamineReach from here →
  1. transport of cysteine and histidine (cytosol to extracellular)
  2. L-histidine carboxy-lyase (histamine-forming)

  Catalysed by SLC7A6, HDCextracellular space, cytosolOne of 3 routes

• L-thyroxineReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and thyroxine (cytosol to extracellular)

  Catalysed by SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• kynurenateReach from here →
  1. L-Cysteine:2-oxoglutarate aminotransferase
  2. 2-oxo-3-sulfanylpropanoate + L-kynurenine = kynurenate + L-cysteine + H2O

  Catalysed by GOT2, GOT1, AADATcytosol

• 5-guanidino-2-oxopentanoateReach from here →
  1. transport of arginine and cysteine (cytosol to extracellular)
  2. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by SLC7A6, IL4I1extracellular space, cytosol

• (3Z)-dodecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

  Catalysed by NAGS, HADHA, ACAA2, HADHBmitochondrial matrixOne of 3 routes

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

  Catalysed by SLC7A5, IDO1, TDO2, IDO2extracellular space, cytosol

• (3S)-citramalyl-CoAReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by CTH, CLYBLcytosol

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

  Catalysed by NAGS, SLC27A2, SLC27A5mitochondrial matrix, endoplasmic reticulumOne of 3 routes

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

  Catalysed by SLC3A2, SLC7A5, SLC7A10, PPATextracellular space, cytosolOne of 2 routes

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

  Catalysed by SLC3A2, SLC7A5, SLC7A10, GFPT2 and 1 moreextracellular space, cytosol

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

  Catalysed by SLC7A5, MSRAextracellular space, cytosol

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

  Catalysed by SLC7A5, MSRB3, MSRB1, MSRB2extracellular space, cytosol

• 5-amino-2-oxopentanoateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by CTH, AGXT2cytosolOne of 3 routes

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

  Catalysed by NAGS, SLC27A2, SLC27A5mitochondrial matrix, cytosolOne of 4 routes

• S-sulfanylglutathioneReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. Sulfide:glutathione,quinone oxidoreductase

  Catalysed by CTH, SQORcytosol, mitochondrial matrixOne of 4 routes

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

  Catalysed by SLC3A2, SLC7A5, SLC7A8, GLYATL2extracellular space, cytosolOne of 2 routes

• 4-aminobutanoateReach from here →
  1. transport of chloride and cysteine (cytosol to extracellular)
  2. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by SLC6A14, SLC6A12extracellular space, cytosolOne of 4 routes

• 3-iodo-L-tyrosineReach from here →
  1. transport of cysteine and tyrosine (cytosol to extracellular)
  2. thyroid peroxidase (iodine)

  Catalysed by SLC7A5, TPOextracellular space, cytosolOne of 2 routes

• dopamineReach from here →
  1. cystine to cysteine conversion
  2. transport of dopamine and GSH (cytosol to extracellular)

  Catalysed by SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

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

  Catalysed by SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• a 2,3-dehydroacyl-CoAReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. a 2,3-saturated acyl-CoA + A = a 2,3-dehydroacyl-CoA + AH2

  Catalysed by MOCOS, ACAD10

• L-prolineReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by SLC7A5, SLC1A4extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin E2Reach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and prostaglandin E2 (cytosol to extracellular)

  Catalysed by SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• N-benzoylglycineReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. Hippurate (Benzoylglycinate) Formation

  Catalysed by SLC3A2, SLC7A5, SLC7A8extracellular space, cytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Carnitine O-Palmitoyltransferase

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 11 routes

• L-cysteinylglycineReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. gamma-glutamyltransferase (alanine)

  Catalysed by SLC7A5, GGTLC3, GGT1, GGT5 and 3 moreextracellular space, cytosolOne of 6 routes

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

  Catalysed by MOCOS, COX15

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

  Catalysed by PPCS, PPCDCcytosol

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by CTH, HOGA1cytosol

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

  Catalysed by NAGS, CPT1A, CPT1Bmitochondrial matrix, cytosol

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

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

  Catalysed by CTH, SQORcytosolOne of 2 routes

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 10 routes

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 9 routes

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. transport of arginine and cysteine (cytosol to extracellular)
  2. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by SLC7A6, NOS3, NOS1, NOS2extracellular space, cytosol

• Mo-molybdopterinReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by SLC7A5, MOCOSextracellular space, cytosolOne of 3 routes

• (2E)-octadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Carnitine O-Palmitoyltransferase

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes