L-cysteine

Three steps

• (9Z,12Z)-octadecadienoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + H2O = (9Z,12Z)-octadecadienoate + CoA + H(+)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosol

• 3-acetamidopropanalReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N1,N12-diacetylspermine to N1-acetylspermine conversion
  3. N1-acetylspermine:oxygen oxidoreductase (3-acetamidopropanal-forming)

  Catalysed by NAGS, PAOX, SMOXmitochondrial matrix, cytosolOne of 4 routes

• oxalateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. hydrogen sulfide + 2 O2 = sulfate + H(+)
  3. oxalate(in) + sulfate(out) = oxalate(out) + sulfate(in)

  Catalysed by CTH, SOD1, SLC25A14, SLC26A2 and 3 morecytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• anthranilic acidReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. L-Kynurenine hydrolase

  Catalysed by SLC7A5, KYNUextracellular space, cytosol

• citric acidReach from here →
  1. L-cysteine hydrogen-sulfide-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 12 routes

• maleateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. hydrogen sulfide + 2 O2 = sulfate + H(+)
  3. maleate(in) + sulfate(out) = maleate(out) + sulfate(in)

  Catalysed by CTH, SOD1, SLC25A14, SLC25A30cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 10 moremitochondrial matrix, cytosolOne of 2 routes

• (9Z)-octadecenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol, endoplasmic reticulumOne of 6 routes

• adipic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Activation of adipic acid for Formation of Adipoyl Carnitine
  3. Thioesterification of adipoyl-CoA for Release into Cytosol

  Catalysed by NAGS, ACOT8mitochondrial matrix, cytosol, peroxisomeOne of 3 routes

• (S)-dihydroorotateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. a quinone + hydrogen sulfide + glutathione + H(+) = S-sulfanylglutathione + a quinol
  3. (S)-dihydroorotate + a quinone = orotate + a quinol

  Catalysed by CTH, SQOR, DHODHcytosol

• an alcoholReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  3. a hydroperoxide + 2 glutathione = an alcohol + glutathione disulfide + H2O

  Catalysed by CTH, TSTD1, CP, PRDX6cytosol

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

  Catalysed by CTH, PKM, PKLR, SLC25A1cytosol, mitochondrial matrixOne of 8 routes

• glutarateReach from here →
  1. L-Cysteine:2-oxoglutarate aminotransferase
  2. 2-oxo-3-sulfanylpropanoate + L-kynurenine = kynurenate + L-cysteine + H2O
  3. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)

  Catalysed by GOT2, GOT1, AADAT, SLC22A6cytosol, inner side of the membrane, outer side of the membraneOne of 15 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. L-cysteine hydrogen-sulfide-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-cysteine hydrogen-sulfide-lyase (deaminating
  2. hydrogen sulfide + 2 O2 = sulfate + H(+)
  3. (2R,3R)-tartrate(in) + sulfate(out) = (2R,3R)-tartrate(out) + sulfate(in)

  Catalysed by CTH, SOD1, SLC25A14, SLC25A30cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (S,S)-tartrateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. hydrogen sulfide + 2 O2 = sulfate + H(+)
  3. (S,S)-tartrate(in) + sulfate(out) = (S,S)-tartrate(out) + sulfate(in)

  Catalysed by CTH, SOD1, SLC25A14, SLC25A30cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3R)-citramalateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. hydrogen sulfide + 2 O2 = sulfate + H(+)
  3. (3R)-citramalate(in) + sulfate(out) = (3R)-citramalate(out) + sulfate(in)

  Catalysed by CTH, SOD1, SLC25A14, SLC25A30cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3S)-citramalateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. hydrogen sulfide + 2 O2 = sulfate + H(+)
  3. (3S)-citramalate(in) + sulfate(out) = (3S)-citramalate(out) + sulfate(in)

  Catalysed by CTH, SOD1, SLC25A14, SLC25A30cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• docosan-1-olReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. docosanoyl-CoA + 2 NADPH + 2 H(+) = docosan-1-ol + 2 NADP(+) + CoA

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

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosolOne of 4 routes

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

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

• octadecan-1-olReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + 2 NADPH + 2 H(+) = octadecan-1-ol + 2 NADP(+) + CoA

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

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 8 moremitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol, endoplasmic reticulumOne of 8 routes

• (15Z)-tetracosenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (nervonic acid)
  3. (15Z)-tetracosenoyl-CoA hydrolase

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

• (11Z)-eicosenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)
  3. (11Z)-eicosenoyl-CoA hydrolase

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

• cis-aconitic acidReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. pyruvate kinase (dTDP)
  3. transport of cis-aconitate and PEP (cytosol to mitochondria)

  Catalysed by CTH, PKM, PKLR, SLC25A1cytosol, mitochondrial matrixOne of 8 routes

• N-carbamoyl-L-aspartateReach from here →
  1. L-Cysteine:oxygen oxidoreductase
  2. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)
  3. carbamoyl phosphate + L-aspartate = N-carbamoyl-L-aspartate + phosphate + H(+)

  Catalysed by CDO1, SLC25A12, SLC25A13, CADcytosol, mitochondrial matrixOne of 2 routes

• methylarsonateReach from here →
  1. thioredoxin-disulfide reductase (NADPH) (cystine)
  2. cystine to cysteine conversion
  3. methylarsonate + 2 glutathione + H(+) = methylarsonous acid + glutathione disulfide + H2O

  Catalysed by TXNRD1, TXNRD3, TXNRD2, GSTO1 and 1 morecytosol

• 2-oxohexanoateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by CTH, AGXT2cytosol

• an acylcholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Acetyl-CoA:choline O-acetyltransferase
  3. an acylcholine + H2O = a carboxylate + choline + H(+)

  Catalysed by NAGS, CHAT, BCHEmitochondrial matrix, nucleus

• fatty acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (elaidate)
  3. (10Z)-heptadecenoic acid formation

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 8 routes

• an organic disulfideReach 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. L-cysteinyl-[sulfatase] + 2 a thiol + O2 = an organic disulfide + 3-oxo-L-alanyl-[sulfatase] + hydrogen sulfide + H2O + H(+)

  Catalysed by CTH, SUMF1cytosol

• L-alpha-aminobutyric acidReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. glutamic-pyruvic transaminase ((S)-2-aminobutanoate)

  Catalysed by CTH, AGXT2, GPTcytosol

• chenodeoxycholateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Propanoyl-CoA:acetyl-CoA C-acyltransferase
  3. chenodeoxycholoyl-CoA hydrolase

  Catalysed by NAGS, ACAA1, SCP2, MYO5B and 3 moremitochondrial matrix, peroxisomeOne of 3 routes

• taurodeoxycholateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by NAGS, SLC27A2, SLC27A5, BAATmitochondrial matrix, cytosolOne of 4 routes

• 3-hydroxyanthranilateReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by SLC7A5, KYNUextracellular space, cytosol

• N-formylanthranilic acidReach from here →
  1. transport of cysteine and tryptophan (cytosol to extracellular)
  2. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)
  3. Formylkynurenine hydrolase

  Catalysed by SLC7A5, IDO1, TDO2, IDO2 and 1 moreextracellular space, cytosol

• cholesteryl hexadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. Acyl-CoA:cholesterol O-acyltransferase

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

• a 1-O-alkyl-2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-alkyl-sn-glycero-3-phosphocholine O-acyltransferase

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 5 moremitochondrial matrix, Golgi apparatus, cytosol

• CHEBI:36882Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Itaconate:CoA ligase (GDP-forming)
  3. (3S)-citramalyl-CoA hydro-lyase (itaconyl-CoA-forming)

  Catalysed by NAGS, SUCLG1, SUCLG2, SUCLA2 and 1 moremitochondrial matrix

• 3-hydroxyisovaleric acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)
  3. Formation of 3-Hydroxy-Isovalerate

  Catalysed by NAGS, CPT1A, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

• 3,7,11,15-tetramethylhexadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. phytanate-CoA ligase
  3. peroxisomal acyl-CoA thioesterase

  Catalysed by NAGS, ACOT2mitochondrial matrix, cytosol, peroxisomeOne of 3 routes

• sphingosine 1-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. ATP:sphingosine 1-phosphotransferase

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 4 moremitochondrial matrix, Golgi apparatus, cytosolOne of 2 routes

• CHEBI:37946Reach from here →
  1. cystine to cysteine conversion
  2. transport of dopamine and GSH (cytosol to extracellular)
  3. aryl sulfotransferase (dopamine)

  Catalysed by SLCO1A2, SLCO1B3, SULT1A3, SULT1A4 and 2 morecytosol, extracellular space

• cholesteryl (9Z,12Z)-octadecadienoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

• D-glucopyranose 6-phosphateReach from here →
  1. transport of cysteine and glutamine (cytosol to extracellular)
  2. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)
  3. glucose-6-phosphatase (carbamoyl-phosphate)

  Catalysed by SLC3A2, SLC7A5, SLC7A10, CAD and 1 moreextracellular space, cytosol, endoplasmic reticulum

• 25-hydroxycholesterolReach 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. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O

  Catalysed by SLC7A5, MOCOS, CH25Hextracellular space, cytosol

• (3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA

  Catalysed by CTH, HMGCL, HMGCLL1cytosol, mitochondrial matrixOne of 2 routes