L,L-cystathionine

Three steps

• gamma-Glu-AlaReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by CTH, SLC7A5, GGT1, GGT5 and 4 morecytosol, extracellular spaceOne of 2 routes

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

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 2 morecytosol, extracellular space, mitochondrial matrix

• an N-acylsphing-4-enineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + a fatty acyl-CoA = an N-acylsphing-4-enine + CoA + H(+)

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 7 moremitochondrial matrix, Golgi apparatus

• N-tetracosanoylsphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (lignocerate)
  3. tetracosanoyl-CoA + sphinganine = N-tetracosanoylsphinganine + CoA + H(+)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 8 moremitochondrial matrix, cytosol

• N-hexacosanoylsphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + sphinganine = N-hexacosanoylsphinganine + CoA + H(+)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 8 moremitochondrial matrix, cytosol

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

  Catalysed by CTH, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• a 1,2-diacyl-sn-glycero-3-phospho-L-serineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phospho-L-serine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-L-serine + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 4 moremitochondrial matrix, Golgi apparatus

• a 3-acylpyruvateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by CTH, AGXT2, FAHD1cytosol

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

  Catalysed by NAGS, ALDH6A1, MMUTmitochondrial matrixOne of 3 routes

• (2E)-butenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 5 moremitochondrial matrix, cytosolOne of 4 routes

• 3-methyl-(2E)-glutaconyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. glycine-N-acyltransferase (3-methylcrotonoylglycine)
  3. 3-Methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)

  Catalysed by NAGS, GLYAT, MCCC1, MCCC2mitochondrial matrixOne of 2 routes

• 3-oxohexadecanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. tetradecanoyl-CoA + malonyl-CoA + H(+) = 3-oxohexadecanoyl-CoA + CO2 + CoA

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol

• (2E)-glutaconyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Production of Glutaryl Carnitine
  3. glutaryl-CoA + O2 = (2E)-glutaconyl-CoA + H2O2

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 1 moremitochondrial matrix, cytosol

• acryloyl-CoAReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutyrate oxidation
  3. medium-chain acyl-CoA dehydrogenase (propanoyl-CoA)

  Catalysed by CTH, ACADS, ACADSBcytosol, mitochondrial matrix

• (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)
  3. (8Z,11Z,14Z)-eicosatrienoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol

• hexadecanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Carnitine O-Palmitoyltransferase
  3. (2E)-hexadecenoyl-CoA + NADPH + H(+) = hexadecanoyl-CoA + NADP(+)

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 1 moremitochondrial matrix, cytosolOne of 2 routes

• (9Z)-octadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. octadecanoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (9Z)-octadecenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol

• propanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. (S)-methylmalonyl-CoA hydrolase
  3. (S)-methylmalonyl-CoA + H(+) = propanoyl-CoA + CO2

  Catalysed by NAGS, ECHDC1mitochondrial matrix

• prostaglandin E1Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin E1 (cytosol to extracellular)

  Catalysed by CTH, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin I2Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin I2 (cytosol to extracellular)

  Catalysed by CTH, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin F2alphaReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin F2alpha (cytosol to extracellular)

  Catalysed by CTH, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin H2Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin H2 (cytosol to extracellular)

  Catalysed by CTH, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin D2Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin D2 (cytosol to extracellular)

  Catalysed by CTH, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. Glutathione: 15-HPETE oxidoreductase

  Catalysed by CTH, GPX5, GPX1, GPX2 and 6 morecytosol

• D-alanineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

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

• sarcosineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

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

• S-adenosyl 3-(methylsulfanyl)propylamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. S-adenosyl 3-(methylsulfanyl)propylamine + putrescine = S-methyl-5'-thioadenosine + spermidine + H(+)

  Catalysed by NAGS, SAT1, SAT2, SRMmitochondrial matrix, cytosol

• 7,8-dihydrofolateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  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 CTH, MOCOS, MTO1cytosolOne of 2 routes

• 5-formimidoyltetrahydrofolateReach from here →
  1. L-homocysteine + L-serine = L,L-cystathionine + H2O
  2. (6S)-5-methyl-5,6,7,8-tetrahydrofolate + L-homocysteine = (6S)-5,6,7,8-tetrahydrofolate + L-methionine
  3. 5-formimidoyltetrahydrofolate + L-glutamate = N-formimidoyl-L-glutamate + (6S)-5,6,7,8-tetrahydrofolate

  Catalysed by CBS, MTR, FTCD

• leukotriene A4Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. transport of GSH and leukotriene A4 (cytosol to extracellular)

  Catalysed by CTH, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by CTH, SLC7A6, ASLcytosol, extracellular spaceOne of 4 routes

• (R)-S-lactoylglutathioneReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)

  Catalysed by CTH, GLO1, GLOD4cytosolOne of 2 routes

• propane-1,3-diamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. [eIF5A protein]-L-lysine + spermidine = [eIF5A protein]-deoxyhypusine + propane-1,3-diamine

  Catalysed by NAGS, SAT1, SAT2, DHPSmitochondrial matrix, cytosol

• 4-guanidinobutanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

  Catalysed by CTH, SLC7A6, GATMcytosol, extracellular space

• 2-oxoadipateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. L-2-aminoadipate + glyoxylate = 2-oxoadipate + glycine

  Catalysed by CTH, AGXT2, AADATcytosol

• L-dopaReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-dopa(out) + L-phenylalanine(in) = L-dopa(in) + L-phenylalanine(out)

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

• 1-hexadecanoyl-sn-glycero-3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. glycerol-3-phosphate 1-O-acyltransferase (palmitoyl-CoA)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosol

• a 1-acylglycerone 3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. dihydroxyacetone phosphate + an acyl-CoA = a 1-acylglycerone 3-phosphate + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 2 moremitochondrial matrix, Golgi apparatus

• O-acetyl-(R)-carnitineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Carnitine O-Palmitoyltransferase
  3. Acetyl-CoA:carnitine O-acetyltransferase

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 9 routes

• 3-guanidinopropanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

  Catalysed by CTH, SLC7A6, GATMcytosol, extracellular space

• L-histidineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-histidine(in) + L-phenylalanine(out) = L-histidine(out) + L-phenylalanine(in)

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

• a 1,2-diacyl-sn-glycero-3-phosphocholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acyl-sn-glycero-3-phosphocholine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 2 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• D-ornithineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. D-ornithine(in) + L-ornithine(out) = D-ornithine(out) + L-ornithine(in)

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

• an N-acylglycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 3 morecytosol, extracellular spaceOne of 2 routes

• S-formylglutathioneReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. alcohol dehydrogenase (formaldehyde)

  Catalysed by CTH, ADH1C, ADH1A, ADH5 and 3 morecytosol

• 2-methyl-3-oxopropanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by CTH, AGXT2cytosol

• guanidinoacetateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. L-Arginine:glycine amidinotransferase

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

• D-threonineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

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

• hypotaurineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-Cysteine:oxygen oxidoreductase
  3. 3-sulfino-L-alanine + H(+) = hypotaurine + CO2

  Catalysed by CTH, CDO1, GADL1, CSADcytosol

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol)Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phospho-(1D-myo-inositol) + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol) + CoA

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 3 moremitochondrial matrix, Golgi apparatus