N-acetyl-L-cysteine

Three steps

• Gln-tRNA(Gln)Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. L-Glutamine:tRNA(Gln) ligase (AMP-forming)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosol

• isocyanateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. L-citrulline + H(+) = isocyanate + L-ornithine

  Catalysed by NAGS, SLC7A6, LACC1mitochondrial matrix, extracellular space, cytosol

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

  Catalysed by NAGS, CTH, SUMF1mitochondrial matrix, cytosol

• Asn-tRNA(Asn)Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of asparagine and cysteine (cytosol to extracellular)
  3. L-Asparagine:tRNA(Asn) ligase (AMP-forming)

  Catalysed by NAGS, SLC7A5, NARS1mitochondrial matrix, extracellular space, cytosol

• glycocholateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. bile acid-CoA: amino acid N-acyltransferase

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

• cholateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. solute carrier organic anion transporter (cholate)

  Catalysed by NAGS, SLCO1A2, SLCO1B3mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• D-aspartateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)
  3. D-aspartate(out) + K(+)(in) + 3 Na(+)(out) + H(+)(out) = D-aspartate(in) + K(+)(out) + 3 Na(+)(in) + H(+)(in)

  Catalysed by NAGS, SLC1A1, SLC1A7, SLC1A3 and 2 moremitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-aspartateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-Cysteine:oxygen oxidoreductase
  3. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)

  Catalysed by NAGS, CDO1, SLC25A12, SLC25A13mitochondrial matrix, cytosolOne of 26 routes

• acetateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. transport of acetate and pyruvate (cytosol to extracellular)

  Catalysed by NAGS, CTH, SLC16A4, SLC16A5 and 3 moremitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• CHEBI:30249Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)

  Catalysed by NAGS, SLC7A5, IDO1, TDO2 and 1 moremitochondrial matrix, extracellular space, cytosol

• formic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. transport of formate and pyruvate (cytosol to extracellular)

  Catalysed by NAGS, CTH, SLC16A4, SLC16A5 and 3 moremitochondrial matrix, cytosol, extracellular spaceOne of 4 routes

• propionic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. transport of propanoate and pyruvate (cytosol to extracellular)

  Catalysed by NAGS, CTH, SLC16A4, SLC16A5 and 4 moremitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• butyric acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. transport of butyrate and pyruvate (cytosol to extracellular)

  Catalysed by NAGS, CTH, SLC16A4, SLC16A5 and 4 moremitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• malonic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. Malonate Transport, Antiport with Chloride

  Catalysed by NAGS, SLC6A14mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 3-hydroxypyruvic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. L-Serine:pyruvate aminotransferase

  Catalysed by NAGS, CTH, AGXTmitochondrial matrix, cytosolOne of 2 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. transport of arachidonate and GSH (cytosol to extracellular)

  Catalysed by NAGSmitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• L-lysineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and leucine (cytosol to extracellular)
  3. L-lysine(in) + L-leucine(out) + Na(+)(out) = L-lysine(out) + L-leucine(in) + Na(+)(in)

  Catalysed by NAGS, SLC7A9, SLC3A1, SLC7A7mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 5 routes

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

• putrescineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. Putrescine Export via Putrescine/Arginine Antiporter

  Catalysed by NAGS, SLC7A6, SLC3A2, SLC7A7mitochondrial matrix, extracellular space, cytosolOne of 4 routes

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

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

  Catalysed by NAGS, CTH, AGXT2mitochondrial matrix, cytosol

• thiosulfateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-mercaptopyruvate:sulfide sulfurtransferase

  Catalysed by NAGS, GOT2, GOT1, MPST and 1 moremitochondrial matrix, cytosolOne of 4 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by NAGS, SLC7A5, IL4I1mitochondrial matrix, extracellular space, cytosol

• tetra-mu3-sulfido-tetrairon(2+)Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. De novo synthesis of 2Fe2S
  3. Synthesis of 4Fe4S from 2Fe2S

  Catalysed by NAGS, NDUFAB1, FDXR, FXN and 10 moremitochondrial matrix

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

  Catalysed by NAGS, SLCO1A2, SLCO1B3mitochondrial matrix, cytosol, extracellular spaceOne of 3 routes

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and isoleucine (cytosol to extracellular)
  3. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by NAGS, SLC7A5, BCAT2, BCAT1mitochondrial matrix, extracellular space, cytosol

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

• D-serineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. D-serine = pyruvate + NH4(+)

  Catalysed by NAGS, CTH, SRRmitochondrial matrix, cytosolOne of 11 routes

• 5-aminolevulinateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

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

• 3-methyl-2-oxovaleric acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and isoleucine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by NAGS, SLC7A5, BCAT1mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 3-(4-hydroxyphenyl)pyruvateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. L-tyrosine:2-oxoglutarate aminotransferase

  Catalysed by NAGS, SLC7A5, GOT2, GOT1 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

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

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• taurocholateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. solute carrier organic anion transporter (GSH)

  Catalysed by NAGS, SLCO1A2, SLCO1B3mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• glycochenodeoxycholic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol, peroxisomeOne of 3 routes

• glyoxylateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. glycine + 2-oxoglutarate = glyoxylate + L-glutamate

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 11 routes

• glycolithocholic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. bile acid-CoA:amino acid N-acyltransferase (glycine)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 4 routes

• L-2-amino-3-oxobutanoic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• (R)-lactic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. (R)-Lactate:NAD+ oxidoreductase

  Catalysed by NAGS, CTH, LDHDmitochondrial matrix, cytosol

• (S)-lactic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. (S)-Lactate:NAD+ oxidoreductase

  Catalysed by NAGS, CTH, LDHB, LDHAL6Bmitochondrial matrix, cytosolOne of 5 routes

• 2-iminopropanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by NAGS, CTH, RIDAmitochondrial matrix, cytosol

• phosphoenolpyruvic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. pyruvate kinase (dTDP)

  Catalysed by NAGS, CTH, PKM, PKLRmitochondrial matrix, cytosol

• N-acetyl-beta-neuraminic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by NAGS, CTH, NPLmitochondrial matrix, cytosol

• gamma-Glu-AlaReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by NAGS, SLC7A5, GGT1, GGT5 and 4 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

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

• taurineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by NAGS, BAATmitochondrial matrix, cytosolOne of 4 routes

• 3,3',5-triiodo-L-thyronineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. transport of GSH and triiodothyronine (cytosol to extracellular)

  Catalysed by NAGS, SLCO1A2, SLCO1B3mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• a 3-acylpyruvateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by NAGS, CTH, FAHD1mitochondrial matrix, cytosol

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

  Catalysed by NAGS, ALDH6A1mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by NAGSmitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by NAGS, ACP2mitochondrial matrix, lysosomeOne of 2 routes