hydrogen sulfide

Three steps

• a 3-acylpyruvateReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by CBS, CTH, FAHD1cytosol

• (S)-malyl-CoAReach from here →
  1. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  2. transport of malate and thiosulfate (cytosol to mitochondria)
  3. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate

  Catalysed by TSTD1, SLC25A10, SUCLG1, SUCLA2mitochondrial matrix, cytosol

• (R)-methylmalonyl-CoAReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by CBS, NAGS, ALDH6A1cytosol, mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by CBS, NAGScytosol, mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by CBS, NAGS, ACP2cytosol, mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by CBS, NAGScytosol, mitochondrial matrixOne of 3 routes

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

  Catalysed by CBS, NAGS, ACAA1, MYO5Bcytosol, mitochondrial matrix, peroxisomeOne of 2 routes

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

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 7 routes

• butanoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by CBS, NAGS, ACSM4, ACSM1 and 4 morecytosol, mitochondrial matrixOne of 9 routes

• choloyl-CoAReach from here →
  1. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  2. cholate(in) + glutathione(in) + ATP + H2O = cholate(out) + glutathione(out) + ADP + phosphate + H(+)
  3. cholate + ATP + CoA = choloyl-CoA + AMP + diphosphate

  Catalysed by TSTD1, ABCC4, SLC27A5inner side of the membrane, outer side of the membrane

• dodecanoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 9 routes

• glutaryl-CoAReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Production of Glutaryl Carnitine

  Catalysed by CBS, NAGS, CPT1A, CPT1C and 1 morecytosol, mitochondrial matrixOne of 2 routes

• eicosanoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 12 routes

• itaconyl-CoAReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by CBS, NAGS, SUCLG1, SUCLG2 and 1 morecytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 13 routes

• tetradecanoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 6 morecytosol, mitochondrial matrixOne of 11 routes

• octanoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by CBS, NAGS, CROTcytosol, mitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. phytanate-CoA ligase

  Catalysed by CBS, NAGScytosol, mitochondrial matrixOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by CBS, NAGS, SLC27A2, ACSL4 and 5 morecytosol, mitochondrial matrixOne of 15 routes

• prostaglandin E1Reach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin E1 (cytosol to extracellular)

  Catalysed by CBS, SLCO2A1cytosol, extracellular spaceOne of 4 routes

• prostaglandin I2Reach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin I2 (cytosol to extracellular)

  Catalysed by CBS, SLCO2A1cytosol, extracellular spaceOne of 4 routes

• prostaglandin F2alphaReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin F2alpha (cytosol to extracellular)

  Catalysed by CBS, SLCO2A1cytosol, extracellular spaceOne of 6 routes

• prostaglandin H2Reach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin H2 (cytosol to extracellular)

  Catalysed by CBS, SLCO2A1cytosol, extracellular spaceOne of 4 routes

• prostaglandin D2Reach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin D2 (cytosol to extracellular)

  Catalysed by CBS, SLCO2A1cytosol, extracellular spaceOne of 4 routes

• 15-oxo-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  2. (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + 2 glutathione = (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + glutathione disulfide + H2O
  3. (15S)-15-Hydroxy-5,8,11-cis-13-trans-icosatetraenoate:NADP+ 15-oxidoreductase

  Catalysed by TSTD1, GPX1, GPX2, GPX4cytosolOne of 3 routes

• D-alanineReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by CBS, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• sarcosineReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

  Catalysed by CBS, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular spaceOne of 3 routes

• 7,8-dihydrofolateReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  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 CBS, MOCOS, MTO1cytosolOne of 4 routes

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  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 CBS, SLC3A2, SLC7A5, SLC7A8 and 2 morecytosol, extracellular space

• leukotriene A4Reach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. cystine to cysteine conversion
  3. transport of GSH and leukotriene A4 (cytosol to extracellular)

  Catalysed by CBS, SLCO2A1cytosol, extracellular spaceOne of 5 routes

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by CBS, SLC7A6, ASLcytosol, extracellular spaceOne of 8 routes

• 4-guanidinobutanoateReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

  Catalysed by CBS, SLC7A6, GATMcytosol, extracellular space

• L-dopaReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-dopa(out) + L-phenylalanine(in) = L-dopa(in) + L-phenylalanine(out)

  Catalysed by CBS, SLC7A5, SLC7A8cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(6)-(1,2-dicarboxyethyl)-AMPReach from here →
  1. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)
  2. transport of fumarate and thiosulfate (cytosol to mitochondria)
  3. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)

  Catalysed by TSTD1, SLC25A10, ADSL, TNRC6Bmitochondrial matrix, cytosolOne of 4 routes

• 3-guanidinopropanoateReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

  Catalysed by CBS, SLC7A6, GATMcytosol, extracellular space

• L-histidineReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-histidine(in) + L-phenylalanine(out) = L-histidine(out) + L-phenylalanine(in)

  Catalysed by CBS, SLC7A5, SLC7A8cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 7 routes

• dihydroxyacetone phosphateReach from here →
  1. a quinone + hydrogen sulfide + glutathione + H(+) = S-sulfanylglutathione + a quinol
  2. a quinol + 2 Fe(III)-[cytochrome c](out) = a quinone + 2 Fe(II)-[cytochrome c](out) + 2 H(+)(out)
  3. a quinone + sn-glycerol 3-phosphate = dihydroxyacetone phosphate + a quinol

  Catalysed by SQOR, CYC1, UQCRFS1, GPD2outer side of the membrane

• D-ornithineReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. D-ornithine(in) + L-ornithine(out) = D-ornithine(out) + L-ornithine(in)

  Catalysed by CBS, SLC7A6, SLC25A2cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• an N-acylglycineReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by CBS, SLC3A2, SLC7A5, SLC7A8 and 3 morecytosol, extracellular space

• 2-methyl-3-oxopropanoateReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by CBS, CTH, AGXT2cytosol

• guanidinoacetateReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. L-Arginine:glycine amidinotransferase

  Catalysed by CBS, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular spaceOne of 5 routes

• 3',5'-cyclic GMPReach from here →
  1. hydrogen sulfide + 2 O2 = sulfate + H(+)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. 3',5'-cyclic GMP(out) + a dicarboxylate(in) = 3',5'-cyclic GMP(in) + a dicarboxylate(out)

  Catalysed by SOD1, SLC25A35, SLC25A34, SLC22A6inner side of the membrane, outer side of the membraneOne of 2 routes

• sphing-4-enineReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by CBS, NAGS, ASAH1, ACER2 and 2 morecytosol, mitochondrial matrix, Golgi apparatusOne of 2 routes

• D-threonineReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by CBS, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• spermidineReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by CBS, NAGS, SAT1, SAT2cytosol, mitochondrial matrixOne of 3 routes

• hypotaurineReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. L-Cysteine:oxygen oxidoreductase
  3. 3-sulfino-L-alanine + H(+) = hypotaurine + CO2

  Catalysed by CBS, CDO1, GADL1, CSADcytosol

• adenosine 5'-phosphoramidateReach from here →
  1. hydrogen sulfide + 2 O2 = sulfate + H(+)
  2. ATP:sulfate adenylyltransferase
  3. adenosine 5'-phosphosulfate + NH4(+) = adenosine 5'-phosphoramidate + sulfate + 2 H(+)

  Catalysed by SOD1, PAPSS1, PAPSS2, FHITcytosol

• L-dopaquinoneReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,L-dopa:oxygen oxidoreductase

  Catalysed by CBS, SLC7A5, TYRcytosol, extracellular space

• L-threonineReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)

  Catalysed by CBS, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 10 routes

• L-saccharopineReach from here →
  1. L-serine hydro-lyase (adding hydrogen sulfide, L-cysteine-forming)
  2. transport of cysteine and lysine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

  Catalysed by CBS, SLC7A6, AASScytosol, extracellular space, mitochondrial matrix