N-acetyl-L-cysteine

Three steps

• CHEBI:15746Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by NAGS, SAT1, SAT2mitochondrial matrix, cytosolOne of 2 routes

• CHEBI:15765Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

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

• bromideReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. 2-bromoacetaldehyde:glutathione S-(formylmethyl)transferase

  Catalysed by NAGS, MGST3, GSTA4, GSTA1 and 14 moremitochondrial matrix, cytosolOne of 2 routes

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

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

• thioredoxin dithiolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-Cysteine:tRNA(Cys) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by NAGS, CARS1mitochondrial matrix, cytosolOne of 2 routes

• methanethiolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. methanethiol + O2 + H2O = hydrogen sulfide + formaldehyde + H2O2 + H(+)

  Catalysed by NAGS, CTH, SELENBP1mitochondrial matrix, cytosol

• sulfateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. hydrogen sulfide + 2 O2 = sulfate + H(+)

  Catalysed by NAGS, CTH, SOD1mitochondrial matrix, cytosolOne of 2 routes

• ureaReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. L-Arginine amidinohydrolase

  Catalysed by NAGS, SLC7A6, ARG1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• 2-methyl-3-oxopropanoic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by NAGS, CTH, AGXT2mitochondrial matrix, cytosol

• 11-cis-retinolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by NAGS, DGAT1, LRAT, AWAT1 and 2 moremitochondrial matrix, cytosol

• (R)-3-aminoisobutyric acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by NAGS, SLC7A5, AGXT2mitochondrial matrix, extracellular space, cytosol

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

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

• iodideReach 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 3 routes

• oxaloacetateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. Pyruvate:carbon-dioxide ligase (ADP-forming)

  Catalysed by NAGS, CTH, PCmitochondrial matrix, cytosolOne of 5 routes

• nitric oxideReach 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 (Nitric-Oxide-Forming)

  Catalysed by NAGS, SLC7A6, NOS3, NOS1 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• CHEBI:16523Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. transport of D-serine and glycine (cytosol to extracellular)

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

• N(2)-acetyl-L-ornithineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by NAGS, SLC7A6, ACY1mitochondrial matrix, extracellular space, cytosol

• 2-phenylacetamideReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

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

• CHEBI:16633Reach 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

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

  Catalysed by NAGS, SLC7A5, MARS1mitochondrial matrix, extracellular space, cytosol

• (S)-lactateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)

  Catalysed by NAGS, CTH, LDHA, LDHB and 3 moremitochondrial matrix, cytosol

• CHEBI:16668Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-Cysteine:oxygen oxidoreductase
  3. 3-sulfino-L-alanine carboxy-lyase

  Catalysed by NAGS, CDO1, GAD1, CSADmitochondrial matrix, cytosol

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

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

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by NAGS, SLC7A5, CNDP2mitochondrial matrix, extracellular space, cytosol

• 2-oxobutanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate

  Catalysed by NAGS, CTH, AGXT2mitochondrial matrix, cytosolOne of 2 routes

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

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

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

  Catalysed by NAGS, CTH, KYAT1mitochondrial matrix, cytosolOne of 5 routes

• formaldehydeReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. N(6),N(6)-dimethyl-L-lysyl(4)-[histone H3] + 2 A + 2 H2O = L-lysyl(4)-[histone H3] + 2 formaldehyde + 2 AH2

  Catalysed by NAGS, MOCOS, KDM1A, KDM1Bmitochondrial matrixOne of 3 routes

• 3-fumarylpyruvateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by NAGS, CTH, FAHD1mitochondrial matrix, cytosol

• pantetheine 4'-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. (R)-4'-phosphopantothenate:L-cysteine ligase
  3. N-[(R)-4'-Phosphopantothenoyl]-L-cysteine carboxy-lyase

  Catalysed by NAGS, PPCS, PPCDCmitochondrial matrix, cytosolOne of 2 routes

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

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

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

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

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

  Catalysed by NAGS, SLC6A14, SLC26A6mitochondrial matrix, extracellular space, cytosol

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

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

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

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

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

  Catalysed by NAGS, CTH, AGXTmitochondrial matrix, cytosol

• CHEBI:17208Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. glutathione:dehydroascorbate oxidoreductase

  Catalysed by NAGSmitochondrial matrix, cytosol

• homocysteineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. Formation of Homocystine

  Catalysed by NAGSmitochondrial matrix, cytosol

• L-glutamic 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-oxo-acid aminotransferase

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

• anilineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by NAGS, NAT2, NAT1mitochondrial matrix, cytosol

• aceneuramateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by NAGS, CTH, NPLmitochondrial matrix, cytosol

• CHEBI:17312Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by NAGSmitochondrial matrix, cytosolOne of 2 routes

• 5'-deoxyadenosineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O

  Catalysed by NAGS, CTH, TYW1B, TYW1mitochondrial matrix, cytosol

• all-trans-retinolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by NAGS, MOCOS, RETSATmitochondrial matrix

• CHEBI:17380Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by NAGS, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by NAGS, SLC7A5, PRODH2mitochondrial matrix, extracellular space, cytosolOne of 2 routes

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

  Catalysed by NAGS, SLC7A6mitochondrial matrix, extracellular space, cytosol

• AH2Reach 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 24 routes

• 5-aminolevulinic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

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

• indole-3-pyruvateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by NAGS, SLC7A5, IL4I1mitochondrial matrix, extracellular space, cytosolOne of 2 routes