N-acetyl-L-cysteine

Three steps

• (2E)-dodecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by NAGSmitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by NAGS, ACAA1, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

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

• butanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 9 routes

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

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

• glutaryl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Production of Glutaryl Carnitine

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 12 routes

• itaconyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by NAGS, SUCLG1, SUCLG2, SUCLA2mitochondrial matrixOne of 2 routes

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 13 routes

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

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

• octanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by NAGS, CROTmitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. phytanate-CoA ligase

  Catalysed by NAGSmitochondrial matrix, cytosolOne of 5 routes

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 4 moremitochondrial matrix, cytosolOne of 15 routes

• prostaglandin E1Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin E1 (cytosol to extracellular)

  Catalysed by NAGS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• prostaglandin I2Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin I2 (cytosol to extracellular)

  Catalysed by NAGS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• prostaglandin F2alphaReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin F2alpha (cytosol to extracellular)

  Catalysed by NAGS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• prostaglandin H2Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin H2 (cytosol to extracellular)

  Catalysed by NAGS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• prostaglandin D2Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin D2 (cytosol to extracellular)

  Catalysed by NAGS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. Glutathione: 15-HPETE oxidoreductase

  Catalysed by NAGS, GPX5, GPX1, GPX2 and 6 moremitochondrial matrix, cytosol

• D-alanineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by NAGS, SLC7A5, SLC7A10mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• sarcosineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 7,8-dihydrofolateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)

  Catalysed by NAGS, MOCOS, MTO1mitochondrial matrix

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 2 moremitochondrial matrix, extracellular space, cytosol

• leukotriene A4Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. transport of GSH and leukotriene A4 (cytosol to extracellular)

  Catalysed by NAGS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by NAGS, SLC7A6, ASLmitochondrial matrix, extracellular space, cytosolOne of 4 routes

• (R)-S-lactoylglutathioneReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)

  Catalysed by NAGS, GLO1, GLOD4mitochondrial matrix, cytosolOne of 2 routes

• 4-guanidinobutanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

  Catalysed by NAGS, SLC7A6, GATMmitochondrial matrix, extracellular space, cytosol

• L-dopaReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-dopa(out) + L-phenylalanine(in) = L-dopa(in) + L-phenylalanine(out)

  Catalysed by NAGS, SLC7A5, SLC7A8mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 3-guanidinopropanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

  Catalysed by NAGS, SLC7A6, GATMmitochondrial matrix, extracellular space, cytosol

• L-histidineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-histidine(in) + L-phenylalanine(out) = L-histidine(out) + L-phenylalanine(in)

  Catalysed by NAGS, SLC7A5, SLC7A8mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 7 routes

• D-ornithineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. D-ornithine(in) + L-ornithine(out) = D-ornithine(out) + L-ornithine(in)

  Catalysed by NAGS, SLC7A6, SLC25A2mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• an N-acylglycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 3 moremitochondrial matrix, extracellular space, cytosol

• S-formylglutathioneReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. alcohol dehydrogenase (formaldehyde)

  Catalysed by NAGS, ADH1C, ADH1A, ADH5 and 3 moremitochondrial matrix, cytosol

• 2-methyl-3-oxopropanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by NAGS, CTH, AGXT2mitochondrial matrix, cytosol

• guanidinoacetateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. L-Arginine:glycine amidinotransferase

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 5 routes

• sphing-4-enineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. acyl-CoA:sphingosine N-acyltransferase

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

• D-threonineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by NAGS, SLC7A5, SLC7A10mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• spermidineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by NAGS, SAT1, SAT2mitochondrial matrix, cytosolOne of 3 routes

• hypotaurineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-Cysteine:oxygen oxidoreductase
  3. 3-sulfino-L-alanine + H(+) = hypotaurine + CO2

  Catalysed by NAGS, CDO1, GADL1, CSADmitochondrial matrix, cytosol

• L-dopaquinoneReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,L-dopa:oxygen oxidoreductase

  Catalysed by NAGS, SLC7A5, TYRmitochondrial matrix, extracellular space, cytosol

• glutathioneReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by NAGS, CTH, TSTD1mitochondrial matrix, cytosolOne of 3 routes

• L-threonineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)

  Catalysed by NAGS, SLC7A5, SLC7A10mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 8 routes

• L-saccharopineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and lysine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

  Catalysed by NAGS, SLC7A6, AASSmitochondrial matrix, extracellular space, cytosol

• L-kynurenineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by NAGS, SLC7A5, KYNUmitochondrial matrix, extracellular space, cytosolOne of 5 routes

• beta-alanineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)

  Catalysed by NAGS, SLC7A5, SLC7A10mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• leukotriene C4Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. leukotriene C4 hydrolysis

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8mitochondrial matrix, extracellular space, cytosolOne of 5 routes

• taurocyamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by NAGS, SLC7A6, GATMmitochondrial matrix, extracellular space, cytosol

• L-glutamate 5-semialdehydeReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate

  Catalysed by NAGS, SLC7A6, OATmitochondrial matrix, extracellular space, cytosol

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

  Catalysed by NAGS, SLC7A5, KYNUmitochondrial matrix, extracellular space, cytosol

• L,L-cystathionineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by NAGSmitochondrial matrix