N-acetyl-L-cysteine

Three steps

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by NAGS, HADHA, ACADVL, HADHBmitochondrial matrix

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

  Catalysed by NAGS, SLC7A5, AARS1mitochondrial matrix, extracellular space, cytosol

• glycine betaineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. transport of betaine and chloride (cytosol to extracellular)

  Catalysed by NAGS, SLC6A14, SLC6A12mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• trans-urocanateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and histidine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

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

• 5-hydroxy-L-tryptophanReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. (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

  Catalysed by NAGS, SLC7A5, TPH1, TPH2mitochondrial matrix, extracellular space, cytosol

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

  Catalysed by NAGS, SLC7A9, SLC3A1, BCAT2mitochondrial matrix, extracellular space, cytosolOne of 4 routes

• CHEBI:17927Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by NAGSmitochondrial matrix, cytosolOne of 2 routes

• 3-phenylpyruvateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by NAGS, SLC7A5, GOT2, TATmitochondrial matrix, extracellular space, cytosolOne of 5 routes

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

  Catalysed by NAGS, GOT2, GOT1, MPST and 1 moremitochondrial matrix, cytosol

• dTDPReach 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

• CHEBI:18095Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. Transport of Hydroxy-Proline

  Catalysed by NAGS, SLC7A5, SLC1A4mitochondrial matrix, extracellular space, cytosolOne of 5 routes

• CHEBI:18183Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-glutamate:L-cysteine gamma-ligase (ADP-forming)
  3. gamma-glutamylcyclotransferase (gamma-glutamyl-cysteine)

  Catalysed by NAGS, GCLC, GCLM, GGCT and 1 moremitochondrial matrix, cytosol

• N-carbamoyl-beta-alanineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

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

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

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

• 2-phenylethylamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

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

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

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

• ophthalmateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

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

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

  Catalysed by NAGS, SLC7A5, SLC7A8mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 6 routes

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

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

  Catalysed by NAGS, CTH, AGXT2mitochondrial matrix, cytosol

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

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

• 5-(3,3'-dimethylguanidino)-2-oxopentanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by NAGS, CTH, AGXT2mitochondrial matrix, cytosol

• 5-(3-methylguanidino)-2-oxopentanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. N(omega)-methyl-L-arginine + pyruvate = 5-(3-methylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by NAGS, CTH, AGXT2mitochondrial matrix, cytosol

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

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

• (5Z)-dodecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by NAGS, HADHA, ACAA2, HADHBmitochondrial matrixOne of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach 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 (cis-cetoleic acid)

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

• (7Z)-tetradecenoyl-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 ((7Z)-tetradecenoic acid)

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

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

  Catalysed by NAGS, HADHA, ACADVL, HADHBmitochondrial matrixOne of 3 routes

• (7Z,10Z)-hexadecadienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by NAGS, HADHA, ACADVL, HADHBmitochondrial matrix

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by NAGS, ECHS1, ACAA2, HADHmitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by NAGS, ECHS1, ACAA2, HADHmitochondrial matrixOne of 2 routes

• CHEBI:240943Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 3 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• a quinolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. a quinone + hydrogen sulfide + glutathione + H(+) = S-sulfanylglutathione + a quinol

  Catalysed by NAGS, CTH, SQORmitochondrial matrix, cytosol

• hypochlorous acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. Peroxidase

  Catalysed by NAGS, SLC6A14, LPOmitochondrial matrix, extracellular space, cytosolOne of 4 routes

• potassium atomReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. transport of chloride (cytosol to extracellular)

  Catalysed by NAGS, SLC6A14, SLC12A2, SLC12A1mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• phenylacetylglycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

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

• CHEBI:27540Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 6 routes

• elaidic acidReach 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 (elaidate)

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

• 5-hydroxytryptophanReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-Tryptophan, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)

  Catalysed by NAGS, SLC7A5, TPH1, TPH2mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 5(S)-HETEReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. Glutathione: 5-HPETE oxidoreductase

  Catalysed by NAGS, GPX5, GPX1, GPX2 and 6 moremitochondrial matrix, cytosolOne of 2 routes

• 3-mercaptolactic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-Mercaptolactate:NAD+ oxidoreductase

  Catalysed by NAGS, GOT2, GOT1, LDHB and 3 moremitochondrial matrix, cytosol

• CHEBI:28666Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. LTD4 hydrolysis

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• CHEBI:28701Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by NAGS, ACAA1, SCP2, MYO5Bmitochondrial matrix, peroxisomeOne of 7 routes

• pyridoxine 5'-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

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

• 3-disulfanyl-L-alanineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. thioredoxin-disulfide reductase (NADPH) (cystine)
  3. L-cystine thiocysteine-lyase (deaminating

  Catalysed by NAGS, TXNRD1, TXNRD3, TXNRD2 and 1 moremitochondrial matrix, cytosol

• Ca(2+)Reach 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. Ca(2+)(out) + K(+)(out) + 4 Na(+)(in) = Ca(2+)(in) + K(+)(in) + 4 Na(+)(out)

  Catalysed by NAGS, SLC1A1, SLC24A1, SLC24A5 and 3 moremitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by NAGS, SLC7A5, FARSB, FARSAmitochondrial matrix, extracellular space, cytosol

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

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

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

  Catalysed by NAGS, SLC7A5, WARS1mitochondrial matrix, extracellular space, cytosol

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

  Catalysed by NAGS, SLC7A5, IARS1mitochondrial matrix, extracellular space, cytosol