L-cysteine

Three steps

• dTTPReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. pyruvate kinase (dTDP)
  3. DTTP Nucleotidohydrolase

  Catalysed by CTH, PKM, PKLR, ENTPD3 and 2 morecytosol, extracellular spaceOne of 11 routes

• indole-3-acetaldehydeReach from here →
  1. transport of cysteine and tryptophan (cytosol to extracellular)
  2. L-tryptophan decarboxy-lyase
  3. Tryptamine:oxygen oxidoreductase(deaminating)

  Catalysed by SLC7A5, DDC, AOC1, MAOA and 1 moreextracellular space, cytosolOne of 2 routes

• spermine monoaldehydeReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. diamine N-acetyltransferase (N1-acetylspermine)
  3. primary-amine oxidase (spermine)

  Catalysed by NAGS, SAT1, SAT2, AOC2 and 2 moremitochondrial matrix, cytosolOne of 3 routes

• (R)-3,4-dihydroxymandelaldehydeReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and noradrenaline (cytosol to extracellular)
  3. (R)-noradrenaline + O2 + H2O = (R)-3,4-dihydroxymandelaldehyde + H2O2 + NH4(+)

  Catalysed by SLCO1A2, SLCO1B3, MAOA, MAOBcytosol, extracellular space

• O-octanoyl-(R)-carnitineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Carnitine O-Palmitoyltransferase
  3. solute carrier (L-carnitine)

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 1 moremitochondrial matrix, cytosolOne of 11 routes

• CHEBI:18132Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Acetyl-CoA:choline O-acetyltransferase
  3. ATP:choline phosphotransferase

  Catalysed by NAGS, CHAT, CHKA, CHKBmitochondrial matrix, nucleus, cytosol

• thioredoxin disulfideReach from here →
  1. L-Cysteine:tRNA(Cys) ligase (AMP-forming)
  2. glycyl-tRNA(gly) phosphorylation
  3. 2'-deoxyadenosine 5'-triphosphate:oxydized-thioredoxin 2'-oxidoreductase

  Catalysed by CARS1, TXN, RRM1, RRM2 and 2 morecytosolOne of 12 routes

• dodecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. Lauroyl-CoA hydrolase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosolOne of 2 routes

• N(2)-formyl-N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)
  3. 10-formyltetrahydrofolate:5'-phosphoribosylglycinamide formyltransferase

  Catalysed by SLC3A2, SLC7A5, SLC7A8, GARTextracellular space, cytosol

• arsenic triglutathioneReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. arsenic triglutathione + 3 [thioredoxin]-dithiol + 3 S-adenosyl-L-methionine = trimethylarsine + 3 [thioredoxin]-disulfide + 3 glutathione + 3 S-adenosyl-L-homocysteine + 3 H(+)

  Catalysed by CTH, TSTD1, AS3MTcytosolOne of 3 routes

• N-(4-aminobutyl)-3-aminopropanalReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. primary-amine oxidase (spermidine)

  Catalysed by NAGS, SAT1, SAT2, AOC2 and 2 moremitochondrial matrix, cytosolOne of 3 routes

• CHEBI:18405Reach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate:oxygen oxidoreductase

  Catalysed by SLC7A5, SCLY, PNPOextracellular space, cytosolOne of 3 routes

• hydrogen cyanideReach from here →
  1. L-Cysteine:2-oxoglutarate aminotransferase
  2. 3-mercaptopyruvate sulfurtransferase (hydrogen-cyanide)
  3. thiosulfate:cyanide sulfurtranserase

  Catalysed by GOT2, GOT1, MPST, TSTcytosol, mitochondrial matrixOne of 2 routes

• (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (EPA)
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + O2 = (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoA + H2O2

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol

• gamma-L-glutamyl-(2S)-2-aminobutanoateReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. (2S)-2-aminobutanoate + L-glutamate + ATP = gamma-L-glutamyl-(2S)-2-aminobutanoate + ADP + phosphate + H(+)

  Catalysed by SLC3A2, SLC7A5, SLC7A8, AGXT2 and 1 moreextracellular space, cytosol

• (R)-normetanephrineReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and noradrenaline (cytosol to extracellular)
  3. S-Adenosyl-L-methionine:catechol O-methyltransferase

  Catalysed by SLCO1A2, SLCO1B3, COMT, LRTOMTcytosol, extracellular space

• 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. a 2-acyl-sn-glycero-3-phosphoethanolamine + octadecanoyl-CoA = 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosol

• 2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)

  Catalysed by CTH, AGXT2, BCKDHA, BCKDHBcytosol, mitochondrial matrix

• N-methyltryptamineReach from here →
  1. transport of cysteine and tryptophan (cytosol to extracellular)
  2. L-tryptophan decarboxy-lyase
  3. S-Adenosyl-L-methionine:amine N-methyltransferase

  Catalysed by SLC7A5, DDC, INMTextracellular space, cytosol

• D-isoleucineReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. D-serine = pyruvate + NH4(+)
  3. D-isoleucine(out) + D-serine(in) = D-isoleucine(in) + D-serine(out)

  Catalysed by CTH, SRR, SLC7A10cytosol, inner side of the membrane, outer side of the membrane

• 15-deoxy-Delta(12,14)-prostaglandin J2-S-(R)-glutathioneReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. 15-deoxy-Delta(12,14)-prostaglandin J2 + glutathione = 15-deoxy-Delta(12,14)-prostaglandin J2-S-(R)-glutathione

  Catalysed by CTH, TSTD1, MGST3cytosol

• Xaa-L-Pro dipeptideReach 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)
  3. Xaa-L-Pro dipeptide + H2O = an L-alpha-amino acid + L-proline

  Catalysed by SLC7A5, SLC1A4, PEPDextracellular space, cytosol, inner side of the membrane, outer side of the membrane

• 11-cis-retinyl esterReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol + a fatty acyl-CoA = 11-cis-retinyl ester + CoA

  Catalysed by NAGS, DGAT1, LRAT, AWAT1 and 2 moremitochondrial matrix, cytosol

• (6R)-10-formyltetrahydrofolateReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine
  3. (6S)-5,6,7,8-tetrahydrofolate + formate + ATP = (6R)-10-formyltetrahydrofolate + ADP + phosphate

  Catalysed by SLC3A2, SLC7A5, SLC7A8, SHMT1 and 3 moreextracellular space, cytosol

• (5S)-hydroxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (5S)-hydroperoxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoate + 2 glutathione = (5S)-hydroxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoate + glutathione disulfide + H2O

  Catalysed by CTH, TSTD1, GPX1, GPX4cytosol

• (12S)-hydroxy-(5Z,8Z,10E,14Z,17Z)-eicosapentaenoateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (12S)-hydroperoxy-(5Z,8Z,10E,14Z,17Z)-eicosapentaenoate + 2 glutathione = (12S)-hydroxy-(5Z,8Z,10E,14Z,17Z)-eicosapentaenoate + glutathione disulfide + H2O

  Catalysed by CTH, TSTD1, GPX1, GPX4cytosol

• (15S)-hydroxy-(11Z,13E)-eicosadienoateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (15S)-hydroperoxy-(11Z,13E)-eicosadienoate + 2 glutathione = (15S)-hydroxy-(11Z,13E)-eicosadienoate + glutathione disulfide + H2O

  Catalysed by CTH, TSTD1, GPX1, GPX4cytosol

• (17S)-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. (17S)-hydroperoxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoate + 2 glutathione = (17S)-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoate + glutathione disulfide + H2O

  Catalysed by CTH, TSTD1, GPX1, GPX4cytosol

• a hydroxy-1,2-diacyl-glycero-3-phosphocholineReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. a hydroperoxy-1,2-diacyl-glycero-3-phosphocholine + 2 glutathione = a hydroxy-1,2-diacyl-glycero-3-phosphocholine + glutathione disulfide + H2O

  Catalysed by CTH, TSTD1, GPX4cytosol

• biocytinReach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. L-lysine(in) + L-leucine(out) + Na(+)(out) = L-lysine(out) + L-leucine(in) + Na(+)(in)
  3. biocytin + H2O = biotin + L-lysine

  Catalysed by SLC7A9, SLC3A1, SLC7A7, BTDextracellular space, cytosol, inner side of the membrane, outer side of the membrane

• glycodeoxycholate 3-sulfateReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. Glycodeoxycholate amidohydrolase
  3. glycodeoxycholate + 3'-phosphoadenylyl sulfate = glycodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLC3A2, SLC7A5, SLC7A8, SULT2A1extracellular space, cytosol

• 3',5'-diiodo-L-thyronineReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3',5'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5'-triiodo-L-thyronine + AH2

  Catalysed by SLC7A5, MOCOS, DIO3extracellular space, cytosol

• 1-butanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Butanoate:CoA ligase (AMP-forming)
  3. 2-(9Z-octadecenoyl)-glycerol + butanoyl-CoA = 1-butanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 5 moremitochondrial matrix, cytosol

• 1-octanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. 2-(9Z-octadecenoyl)-glycerol + octanoyl-CoA = 1-octanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by NAGS, CROT, MOGAT2, MOGAT1mitochondrial matrix

• 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + 2-(9Z-octadecenoyl)-glycerol = 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol

• (6R)-5,10-methylenetetrahydrofolic acidReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by SLC3A2, SLC7A5, SLC7A8, SHMT1extracellular space, cytosolOne of 8 routes

• O-butanoyl-(R)-carnitineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + (R)-carnitine = O-butanoyl-(R)-carnitine + CoA

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 4 moremitochondrial matrix, cytosol

• O-[(5Z),(8Z),(11Z),(14Z)-eicosatetraenoyl]-beta-D-glucuronateReach from here →
  1. cystine to cysteine conversion
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + UDP-alpha-D-glucuronate = O-[(5Z),(8Z),(11Z),(14Z)-eicosatetraenoyl]-beta-D-glucuronate + UDP

  Catalysed by UGT1A9, UGT2B7, UGT1A6, UGT1A1 and 2 morecytosol, extracellular space

• 20-O-(beta-D-glucuronosyl)-(5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. cystine to cysteine conversion
  2. Resistance-Nodulation-Cell Division (Rnd) Tcdb:2.A.60.1.2
  3. 20-hydroxy-(5Z,8Z,11Z,14Z)-eicosatetraenoate + UDP-alpha-D-glucuronate = 20-O-(beta-D-glucuronosyl)-(5Z,8Z,11Z,14Z)-eicosatetraenoate + UDP + H(+)

  Catalysed by SLCO2A1, UGT2B7, UGT1A1, UGT1A4cytosol, extracellular space

• 15-O-(beta-D-glucuronosyl)-prostaglandin B1Reach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and prostaglandin B1 (cytosol to extracellular)
  3. prostaglandin B1 + UDP-alpha-D-glucuronate = 15-O-(beta-D-glucuronosyl)-prostaglandin B1 + UDP + H(+)

  Catalysed by SLCO2A1, UGT1A9, UGT2B7, UGT1A1 and 1 morecytosol, extracellular space

• (S)-lactoyl-AMPReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (S)-lactate + ATP + H(+) = (S)-lactoyl-AMP + diphosphate

  Catalysed by CTH, LDHA, LDHB, LDHC and 4 morecytosol

• (S)-lactoyl-CoAReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (S)-lactate + ATP + CoA = (S)-lactoyl-CoA + AMP + diphosphate

  Catalysed by CTH, LDHA, LDHB, LDHC and 3 morecytosol

• 3-iodothyronamineReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3-iodothyronamine + iodide + A + H(+) = 3,5-diiodothyronamine + AH2

  Catalysed by SLC7A5, MOCOS, DIO1, DIO3extracellular space, cytosolOne of 2 routes

• N-acetylneuraminate 9-phosphateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. pyruvate kinase (dTDP)
  3. N-acetylneuraminate synthase

  Catalysed by CTH, PKM, PKLR, NANScytosol

• 3-iodo-L-thyronineReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3-iodo-L-thyronine + iodide + A + H(+) = 3,5-diiodo-L-thyronine + AH2

  Catalysed by SLC7A5, MOCOS, DIO1, DIO3extracellular space, cytosolOne of 2 routes

• 3'-iodo-L-thyronineReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3'-iodo-L-thyronine + iodide + A + H(+) = 3',5'-diiodo-L-thyronine + AH2

  Catalysed by SLC7A5, MOCOS, DIO1, DIO2extracellular space, cytosolOne of 2 routes

• 3-oxo-(9Z)-eicosenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)
  3. very-long-chain 3-oxoacyl-CoA synthase ((7Z)-octadecenoyl-CoA)

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 7 moremitochondrial matrix, cytosol

• serotonin O-sulfateReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and serotonin (cytosol to extracellular)
  3. serotonin + 3'-phosphoadenylyl sulfate = serotonin O-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by SLCO1A2, SLCO1B3, SULT1A3, SULT1A4cytosol, extracellular space

• (R)-noradrenaline 4'-O-sulfateReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and noradrenaline (cytosol to extracellular)
  3. sulfotransferase (noradrenaline)

  Catalysed by SLCO1A2, SLCO1B3, SULT1A3cytosol, extracellular spaceOne of 2 routes

• L-thyronineReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. L-thyronine + iodide + A + H(+) = 3-iodo-L-thyronine + AH2

  Catalysed by SLC7A5, MOCOS, DIO3extracellular space, cytosol