N-acetyl-L-cysteine

Three steps

• 6-amino-2-oxohexanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and lysine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by NAGS, SLC7A6mitochondrial matrix, extracellular space, cytosol, peroxisome

• L-homocysteineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by NAGS, SLC7A5, BHMT2mitochondrial matrix, extracellular space, cytosol

• carbamoyl phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 4 routes

• S-methyl-L-methionineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by NAGS, SLC7A5, BHMT2mitochondrial matrix, extracellular space, cytosol

• glutathione disulfideReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. thioredoxin-disulfide reductase (NADPH) (cystine)
  3. cystine to cysteine conversion

  Catalysed by NAGS, TXNRD1, TXNRD3, TXNRD2mitochondrial matrix, cytosolOne of 14 routes

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

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

• L-tyrosineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

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

• 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 6 routes

• an acyl-CoAReach 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

• trans-4-hydroxy-L-prolineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by NAGS, SLC7A5, SLC1A4mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by NAGS, SLC7A6, HDCmitochondrial matrix, extracellular space, cytosolOne of 3 routes

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

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

• kynurenateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 2-oxo-3-sulfanylpropanoate + L-kynurenine = kynurenate + L-cysteine + H2O

  Catalysed by NAGS, GOT2, GOT1, AADATmitochondrial matrix, cytosol

• 5-guanidino-2-oxopentanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by NAGS, SLC7A6, IL4I1mitochondrial matrix, extracellular space, cytosol

• (3Z)-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-5-enoyl-CoA)

  Catalysed by NAGS, HADHA, ACAA2, HADHBmitochondrial matrixOne of 3 routes

• N-formyl-L-kynurenineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

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

• (3S)-citramalyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by NAGS, CTH, CLYBLmitochondrial matrix, cytosol

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by NAGS, SLC27A2, SLC27A5mitochondrial matrix, endoplasmic reticulumOne of 3 routes

• 5-phospho-beta-D-ribosylamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• D-glucosamine 6-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A10 and 2 moremitochondrial matrix, extracellular space, cytosol

• L-methionine (S)-S-oxideReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

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

• L-methionine (R)-S-oxideReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by NAGS, SLC7A5, MSRB3, MSRB1 and 1 moremitochondrial matrix, extracellular space, cytosol

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

  Catalysed by NAGS, CTH, AGXT2mitochondrial matrix, cytosolOne of 3 routes

• deoxycholoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by NAGS, SLC27A2, SLC27A5mitochondrial matrix, cytosolOne of 4 routes

• S-sulfanylglutathioneReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. Sulfide:glutathione,quinone oxidoreductase

  Catalysed by NAGS, CTH, SQORmitochondrial matrix, cytosolOne of 4 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

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

• 4-aminobutanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by NAGS, SLC6A14, SLC6A12mitochondrial matrix, extracellular space, cytosolOne of 4 routes

• 3-iodo-L-tyrosineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

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

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

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

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

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

• a 2,3-dehydroacyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  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 NAGS, MOCOS, ACAD10mitochondrial matrix

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

  Catalysed by NAGS, SLC7A5, SLC1A4mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin E2Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin E2 (cytosol to extracellular)

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

• N-benzoylglycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. Hippurate (Benzoylglycinate) Formation

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• decanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoyl-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 (palmitolate)

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

• L-cysteinylglycineReach 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, GGTLC3, GGT1 and 4 moremitochondrial matrix, extracellular space, cytosolOne of 6 routes

• Fe(II)-heme aReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2

  Catalysed by NAGS, MOCOS, COX15mitochondrial matrix

• (R)-4'-phosphopantetheineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. (R)-4'-phosphopantothenate:L-cysteine ligase
  3. N-[(R)-4-phosphopantothenoyl]-L-cysteine + H(+) = (R)-4'-phosphopantetheine + CO2

  Catalysed by NAGS, PPCS, PPCDCmitochondrial matrix, cytosol

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by NAGS, CTH, HOGA1mitochondrial matrix, cytosol

• 3-hydroxyisovaleryl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by NAGS, CPT1A, CPT1Bmitochondrial matrix, cytosol

• CHEBI:63544Reach 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 (adrenic acid)

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

• ubiquinol-10Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. ubiquinone-10 + hydrogen sulfide + sulfite + 2 H(+) = ubiquinol-10 + thiosulfate

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

• hexacosanoyl-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 (cerotic acid)

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

• tetracosanoyl-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 (lignocerate)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 10 routes

• docosanoyl-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 (behenic acid)

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

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by NAGS, SLC7A6, NOS3, NOS1 and 1 moremitochondrial matrix, extracellular space, cytosol

• Mo-molybdopterinReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

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

• (2E)-octadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes