L-cysteine

Three steps

• methylamineReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. N(omega)-methyl-L-arginine + glyoxylate = 5-(3-methylguanidino)-2-oxopentanoate + glycine
  3. N(omega)-methyl-L-arginine + H2O = L-citrulline + methylamine

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

• monodehydro-L-ascorbate radicalReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. (9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)
  3. N-(9Z-octadecenoyl)glycine + 2 L-ascorbate + O2 = N-(9Z-octadecenoyl)-(2S)-hydroxyglycine + 2 monodehydro-L-ascorbate radical + H2O

  Catalysed by SLC3A2, SLC7A5, SLC7A8, GLYATL3 and 2 moreextracellular space, cytosolOne of 14 routes

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine

  Catalysed by SLC3A2, SLC7A5, SLC7A8, AADAT and 1 moreextracellular space, cytosolOne of 4 routes

• pyridoxal 5'-phosphateReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate + O2 = pyridoxal 5'-phosphate + H2O2

  Catalysed by SLC7A5, SCLY, PNPOextracellular space, cytosol

• 2-hydroxy-4-methylvaleric acidReach from here →
  1. transport of cysteine and leucine (cytosol to extracellular)
  2. L-Leucine:2-oxoglutarate aminotransferase
  3. 4-methyl-2-oxopentanoate reduction

  Catalysed by SLC7A9, SLC3A1, BCAT2, SDR42E2 and 17 moreextracellular space, cytosol, mitochondrial matrix

• an L-alpha-amino acidReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. an L-alpha-amino acid(in) + L-kynurenine(out) = an L-alpha-amino acid(out) + L-kynurenine(in)

  Catalysed by SLC7A5, KYNU, SLC7A11extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (2S)-2-phospholactateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (2S)-2-phospholactate + H2O = (S)-lactate + phosphate

  Catalysed by CTH, LDHA, LDHB, LDHC and 3 morecytosolOne of 2 routes

• 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholineReach 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 + a 1-acyl-sn-glycero-3-phosphocholine = 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholine + CoA

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

• estrone 3-sulfateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. hydrogen sulfide + 2 O2 = sulfate + H(+)
  3. estrone 3-sulfate + H2O = estrone + sulfate + H(+)

  Catalysed by CTH, SOD1, STScytosol

• sphing-4-enine 1-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + ATP = sphing-4-enine 1-phosphate + ADP + H(+)

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

• (2E)-tetradecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. trans-tetradec-2-enoyl-CoA reductase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (2E)-decenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. trans-dec-2-enoyl-CoA reductase

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 4 moremitochondrial matrix, cytosolOne of 8 routes

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

  Catalysed by CARS1, TXN, RRM1, RRM2 and 2 morecytosol

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

  Catalysed by CARS1, TXN, RRM1, RRM2 and 2 morecytosolOne of 3 routes

• (2E)-hexenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. medium-chain acyl-CoA ligase (hexanoic acid)
  3. trans-hex-2-enoyl-CoA reductase

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 4 moremitochondrial matrix, cytosolOne of 3 routes

• a cardiolipinReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1'-[1,2-diacyl-sn-glycero-3-phospho],3'-[1-acyl-sn-glycero-3-phospho]-glycerol + an acyl-CoA = a cardiolipin + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 2 moremitochondrial matrix, Golgi apparatus

• (2E)-octenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. trans-oct-2-enoyl-CoA reductase

  Catalysed by NAGS, CROT, MECRmitochondrial matrixOne of 5 routes

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

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

• (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylateReach from here →
  1. transport of alanine and cysteine (cytosol to extracellular)
  2. Transport of Hydroxy-Proline
  3. trans-4-Hydroxy-L-proline:NAD+ 5-oxidoreductase

  Catalysed by SLC7A5, SLC1A4, PYCR1, PYCR3 and 1 moreextracellular space, cytosolOne of 5 routes

• (5-hydroxyindol-3-yl)acetateReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. hydrogen sulfide + 2 O2 = sulfate + H(+)
  3. Transport of 5-Hydroxyindoleacetate

  Catalysed by CTH, SOD1, SLC4A1cytosol, extracellular spaceOne of 2 routes

• 2-deoxy-D-ribose 5-phosphateReach from here →
  1. transport of cysteine and threonine (cytosol to extracellular)
  2. L-threonine acetaldehyde-lyase (glycine-forming)
  3. 2-deoxy-D-ribose 5-phosphate = D-glyceraldehyde 3-phosphate + acetaldehyde

  Catalysed by SLC7A5, DERAextracellular space, cytosol

• leukotriene D4Reach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. leukotriene C4 hydrolysis
  3. leukotriene C4 + H2O = leukotriene D4 + L-glutamate

  Catalysed by SLC3A2, SLC7A5, SLC7A8, GGT1 and 1 moreextracellular space, cytosol

• an all-trans-retinyl esterReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. all-trans-retinol + an acyl-CoA = an all-trans-retinyl ester + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 5 moremitochondrial matrix, Golgi apparatusOne of 8 routes

• dTMPReach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. pyruvate kinase (dTDP)
  3. DTDP Diphosphohydrolase

  Catalysed by CTH, PKM, PKLR, ENTPD3 and 2 morecytosol, extracellular spaceOne of 5 routes

• 18-hydroxy-(5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. cystine to cysteine conversion
  2. transport of arachidonate and GSH (cytosol to extracellular)
  3. cytochrome P450 (arachidonate)

  Catalysed by CYP4F8cytosol, extracellular space, endoplasmic reticulumOne of 2 routes

• 3-demethylubiquinol-10Reach from here →
  1. L-cysteine hydrogen-sulfide-lyase (deaminating
  2. ubiquinone-10 + hydrogen sulfide + sulfite + 2 H(+) = ubiquinol-10 + thiosulfate
  3. 3-demethylubiquinol-10 + S-adenosyl-L-methionine = ubiquinol-10 + S-adenosyl-L-homocysteine + H(+)

  Catalysed by CTH, SQOR, COQ3cytosol

• 1-O-hexadecyl-sn-glycero-3-phosphocholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-O-hexadecyl-2-acetyl-sn-glycero-3-phosphocholine + sphing-4-enine = 1-O-hexadecyl-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine + H(+)

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 2 moremitochondrial matrix, Golgi apparatus

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

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

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

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

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

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

• 1-octadecanoyl-2-acyl-sn-glycero-3-phospho-1D-myo-inositolReach 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-phospho-D-myo-inositol + octadecanoyl-CoA = 1-octadecanoyl-2-acyl-sn-glycero-3-phospho-1D-myo-inositol + CoA

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

• 3-oxoeicosasphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + L-serine + H(+) = 3-oxoeicosasphinganine + CO2 + CoA

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

• a 2,3-saturated acyl-CoAReach 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. a 2,3-saturated acyl-CoA + A = a 2,3-dehydroacyl-CoA + AH2

  Catalysed by SLC7A5, MOCOS, ACAD10extracellular space, cytosolOne of 2 routes

• 3-oxoeicosanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. very-long-chain 3-oxoacyl-CoA synthase (malonyl-CoA)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 11 moremitochondrial matrix, cytosolOne of 2 routes

• N-docosanoylsphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. docosanoyl-CoA + sphinganine = N-docosanoylsphinganine + CoA + H(+)

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

• N-eicosanoylsphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + sphinganine = N-eicosanoylsphinganine + CoA + H(+)

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

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 10 moremitochondrial matrix, cytosol

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

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

• all-trans-retinyl 9Z-octadecenoateReach 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. 1,2,3-tri-(9Z-octadecenoyl)-glycerol + all-trans-retinol = all-trans-retinyl 9Z-octadecenoate + di-(9Z)-octadecenoylglycerol

  Catalysed by MOCOS, RETSAT, PNPLA4, PNPLA2

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

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

• 3-oxotetradecasphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. dodecanoyl-CoA + L-serine + H(+) = 3-oxotetradecasphinganine + CO2 + CoA

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

• xanthurenateReach from here →
  1. transport of cysteine and glycine (cytosol to extracellular)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. 3-hydroxy-L-kynurenine + glyoxylate = xanthurenate + glycine + H2O

  Catalysed by SLC3A2, SLC7A5, SLC7A8, AADAT and 1 moreextracellular space, cytosolOne of 4 routes

• (R)-adrenalineReach from here →
  1. cystine to cysteine conversion
  2. transport of GSH and noradrenaline (cytosol to extracellular)
  3. (R)-noradrenaline + S-adenosyl-L-methionine = (R)-adrenaline + S-adenosyl-L-homocysteine + H(+)

  Catalysed by SLCO1A2, SLCO1B3, PNMTcytosol, extracellular space

• 3-oxooctadecanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. very-long-chain 3-oxoacyl-CoA synthase (malonyl-CoA)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 12 moremitochondrial matrix, cytosolOne of 2 routes

• 3-oxodocosanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. very-long-chain 3-oxoacyl-CoA synthase (eicosanoyl-CoA)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 12 moremitochondrial matrix, cytosolOne of 3 routes

• 3-oxo-(8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (gamma-linolenate)
  3. (6Z,9Z,12Z)-octadecatrienoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(8Z,11Z,14Z)-eicosatrienoyl-CoA + CO2 + CoA

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

• 3-oxo-(8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (stearidonic acid)
  3. (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoA + CO2 + CoA

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

• (8Z,11Z,14Z)-eicosatrienoateReach 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 2 routes

• 1-dodecanoyl-sn-glycerol 3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. glycerol-3-phosphate 1-O-acyltransferase (lauroyl-CoA)

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

• 1-tetradecanoyl-sn-glycerol 3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. glycerol-3-phosphate 1-O-acyltransferase (myristoyl-CoA)

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