L-cysteine

Three steps

• (6R)-L-erythro-6,7-dihydrobiopterinReach from here →
  1. transport of cysteine and tryptophan (cytosol to extracellular)
  2. (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
  3. (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin = (6R)-L-erythro-6,7-dihydrobiopterin + H2O

  Catalysed by SLC7A5, TPH1, TPH2, PCBD1 and 1 moreextracellular space, cytosol

• all-trans-4-oxoretinolReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  3. cytochrome P450 (retinol)

  Catalysed by MOCOS, RETSAT, CYP26A1cytosol

• 13-cis-retinolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol = 13-cis-retinol

  Catalysed by NAGS, DGAT1, LRAT, AWAT1 and 3 moremitochondrial matrix, cytosolOne of 3 routes

• 13-cis-retinalReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. transport of pyruvate and retinoate (cytosol to extracellular)
  3. retinal dehydrogenase (13-cis-retinal)

  Catalysed by CTH, SLC16A4, SLC16A5, SLC16A3 and 5 morecytosol, extracellular space, endoplasmic reticulum

• spermineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosyl 3-(methylsulfanyl)propylamine + spermidine = spermine + S-methyl-5'-thioadenosine + H(+)

  Catalysed by NAGS, SAT1, SAT2, SMSmitochondrial matrix, cytosolOne of 2 routes

• tert-butanolReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. tert-butyl hydroperoxide + 2 glutathione = tert-butanol + glutathione disulfide + H2O

  Catalysed by CTH, TSTD1, CLIC2, GPX1 and 3 morecytosol

• cholesteryl (9Z-octadecenoate)Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Transport of octadecenoyl-CoA into Mitochondrial Matrix
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 2 moremitochondrial matrix, cytosol, endoplasmic reticulum

• N-(acetyl)-sphing-4-enineReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate

  Catalysed by CTH, SLC16A4, SLC16A5, SLC16A3 and 3 morecytosol, extracellular spaceOne of 3 routes

• a 1,3-diacylglycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acylglycerol + an acyl-CoA = a 1,3-diacylglycerol + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 3 moremitochondrial matrix, Golgi apparatus

• N-methylserotoninReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and serotonin (cytosol to extracellular)
  3. S-Adenosyl-L-methionine:amine N-methyltransferase

  Catalysed by SLCO1A2, SLCO1B3, INMTcytosol, extracellular space

• 2-methyl-1-hydroxypropylthiamine diphosphateReach from here →
  1. transport of cysteine and valine (cytosol to extracellular)
  2. L-Valine:2-oxoglutarate aminotransferase
  3. branched chain keto acid dehydrogenase E1 (3-methyl-2-oxobutyrate)

  Catalysed by SLC7A5, BCAT2, BCKDHA, BCKDHBextracellular space, cytosol, mitochondrial matrix

• a 1,2-diacylglycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acylglycerol + an acyl-CoA = a 1,2-diacylglycerol + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 6 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-oneReach from here →
  1. transport of cysteine and methionine (cytosol to extracellular)
  2. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  3. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 4-methylsulfanyl-2-oxobutanoate + formate + 2 H(+)

  Catalysed by SLC7A5, AADAT, ADI1extracellular space, cytosol

• Li(+)Reach from here →
  1. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)
  2. Ca(2+)(out) + K(+)(out) + 4 Na(+)(in) = Ca(2+)(in) + K(+)(in) + 4 Na(+)(out)
  3. transport of Li+ (cytosol to extracellular)

  Catalysed by SLC1A1, SLC24A1, SLC24A5, SLC24A4 and 3 moreinner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 3 routes

• (5-hydroxyindol-3-yl)acetaldehydeReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and serotonin (cytosol to extracellular)
  3. 5-Hydroxytryptamine:oxygen oxidoreductase(deaminating)(flavin-containing)

  Catalysed by SLCO1A2, SLCO1B3, MAOA, MAOBcytosol, extracellular spaceOne of 2 routes

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

  Catalysed by CTH, SUMF1, PNKDcytosol

• all-trans-13,14-dihydroretinolReach 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. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by SLC7A5, MOCOS, RETSATextracellular space, cytosolOne of 2 routes

• 2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-glycerolReach from here →
  1. cystine to cysteine conversion
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. fatty acid amide hydrolase (2-arachidonoylglycerol)

  Catalysed by FAAHcytosol, extracellular space

• an N-acylsphing-4-enineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + a fatty acyl-CoA = an N-acylsphing-4-enine + CoA + H(+)

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 7 moremitochondrial matrix, Golgi apparatus

• N-tetracosanoylsphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (lignocerate)
  3. tetracosanoyl-CoA + sphinganine = N-tetracosanoylsphinganine + CoA + H(+)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 8 moremitochondrial matrix, cytosol

• N-hexacosanoylsphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + sphinganine = N-hexacosanoylsphinganine + CoA + H(+)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 8 moremitochondrial matrix, cytosol

• 1-(beta-D-ribofuranosyl)-1,4-dihydronicotinamideReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. a quinone + hydrogen sulfide + glutathione + H(+) = S-sulfanylglutathione + a quinol
  3. 1-(beta-D-ribofuranosyl)-1,4-dihydronicotinamide + a quinone + H(+) = beta-nicotinamide D-riboside + a quinol

  Catalysed by CTH, SQOR, NQO2cytosol

• malonaldehydeReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and prostaglandin H2 (cytosol to extracellular)
  3. thromboxane A synthase (12(S)-HHT)

  Catalysed by SLCO2A1, TBXAS1cytosol, extracellular spaceOne of 2 routes

• 3,3',5'-triiodo-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. 3,3',5'-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2

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

• a 1,2-diacyl-sn-glycero-3-phospho-L-serineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phospho-L-serine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-L-serine + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 4 moremitochondrial matrix, Golgi apparatus

• acetoacetyl-CoAReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. acetoacetate + ATP + CoA = acetoacetyl-CoA + AMP + diphosphate

  Catalysed by CTH, AACScytosol, mitochondrial matrixOne of 2 routes

• succinyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)
  3. (R)-methylmalonyl-CoA = succinyl-CoA

  Catalysed by NAGS, ALDH6A1, MMUTmitochondrial matrixOne of 3 routes

• (S)-malyl-CoAReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. (S)-malate + NAD(+) = pyruvate + CO2 + NADH
  3. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate

  Catalysed by CTH, ME2, SUCLG1, SUCLA2cytosol

• (2E)-butenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 5 moremitochondrial matrix, cytosolOne of 4 routes

• 3-methyl-(2E)-glutaconyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. glycine-N-acyltransferase (3-methylcrotonoylglycine)
  3. 3-Methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)

  Catalysed by NAGS, GLYAT, MCCC1, MCCC2mitochondrial matrixOne of 2 routes

• 3-oxohexadecanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. tetradecanoyl-CoA + malonyl-CoA + H(+) = 3-oxohexadecanoyl-CoA + CO2 + CoA

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

• (2E)-glutaconyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Production of Glutaryl Carnitine
  3. glutaryl-CoA + O2 = (2E)-glutaconyl-CoA + H2O2

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 1 moremitochondrial matrix, cytosol

• acryloyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Propanoate:CoA ligase (AMP-forming)
  3. medium-chain acyl-CoA dehydrogenase (propanoyl-CoA)

  Catalysed by NAGS, ACSS3, ACSS2, ACSS1 and 2 moremitochondrial matrix, cytosol

• (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)
  3. (8Z,11Z,14Z)-eicosatrienoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosolOne of 2 routes

• benzoyl-CoAReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. Hippurate (Benzoylglycinate) Formation
  3. benzoyl-CoA + glycine = N-benzoylglycine + CoA + H(+)

  Catalysed by SLC3A2, SLC7A5, SLC7A8, GLYATextracellular space, cytosol, mitochondrial matrix

• choloyl-CoAReach from here →
  1. cystine to cysteine conversion
  2. solute carrier organic anion transporter (cholate)
  3. cholate + ATP + CoA = choloyl-CoA + AMP + diphosphate

  Catalysed by SLCO1A2, SLCO1B3, SLC27A5cytosol, extracellular space

• hexadecanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Carnitine O-Palmitoyltransferase
  3. (2E)-hexadecenoyl-CoA + NADPH + H(+) = hexadecanoyl-CoA + NADP(+)

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 1 moremitochondrial matrix, cytosolOne of 3 routes

• (9Z)-octadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (9Z)-octadecenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

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

• propanoyl-CoAReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine
  3. 2-methyl-3-oxopropanoate + NAD(+) + CoA + H2O = propanoyl-CoA + hydrogencarbonate + NADH + H(+)

  Catalysed by CTH, AGXT2, ALDH6A1cytosolOne of 2 routes

• 15-oxoprostaglandin E2Reach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and prostaglandin E2 (cytosol to extracellular)
  3. prostaglandin E2 + NAD(+) = 15-oxoprostaglandin E2 + NADH + H(+)

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

• 15-oxoprostaglandin E1Reach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and prostaglandin E1 (cytosol to extracellular)
  3. (13E)-(15S)-11alpha,15-dihydroxy-9-oxoprost-13-enoate:NADP+ 15-oxidoreductase

  Catalysed by SLCO2A1, CBR3, CBR1, DHRS4cytosol, extracellular spaceOne of 3 routes

• 15-oxoprostaglandin D2Reach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and prostaglandin D2 (cytosol to extracellular)
  3. 15-dehydro-prostaglandin D2 reduction

  Catalysed by SLCO2A1cytosol, extracellular spaceOne of 2 routes

• 15-oxo-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. cystine to cysteine conversion
  2. Glutathione: 15-HPETE oxidoreductase
  3. (15S)-15-Hydroxy-5,8,11-cis-13-trans-icosatetraenoate:NADP+ 15-oxidoreductase

  Catalysed by GPX5, GPX1, GPX2, GPX3 and 5 morecytosolOne of 3 routes

• S-adenosyl 3-(methylsulfanyl)propylamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosyl 3-(methylsulfanyl)propylamine + putrescine = S-methyl-5'-thioadenosine + spermidine + H(+)

  Catalysed by NAGS, SAT1, SAT2, SRMmitochondrial matrix, cytosol

• (12S)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoateReach from here →
  1. cystine to cysteine conversion
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + O2 = (12S)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate

  Catalysed by ALOX5, ALOX15, ALOX12cytosol, extracellular space

• thromboxane A2Reach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and thromboxane B2 (cytosol to extracellular)
  3. thromboxane A2 hydrolysis

  Catalysed by SLCO1B1cytosol, extracellular space, endoplasmic reticulumOne of 4 routes

• (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. cystine to cysteine conversion
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + O2 = (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate

  Catalysed by ALOX15B, ALOX15, ALOX12cytosol, extracellular space

• (5S)-hydroperoxy-(6E,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. cystine to cysteine conversion
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + O2 = (5S)-hydroperoxy-(6E,8Z,11Z,14Z)-eicosatetraenoate

  Catalysed by ALOX5cytosol, extracellular space

• 5-formimidoyltetrahydrofolateReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5-Formiminotetrahydrofolate:L-glutamate N-formiminotransferase

  Catalysed by SLC3A2, SLC7A5, SLC7A8, SHMT1 and 1 moreextracellular space, cytosolOne of 2 routes

• propane-1,3-diamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. [eIF5A protein]-L-lysine + spermidine = [eIF5A protein]-deoxyhypusine + propane-1,3-diamine

  Catalysed by NAGS, SAT1, SAT2, DHPSmitochondrial matrix, cytosol