L,L-cystathionine

Three steps

• glycine betaineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. transport of betaine and chloride (cytosol to extracellular)

  Catalysed by CTH, SLC6A14, SLC6A12cytosol, extracellular spaceOne of 3 routes

• CHEBI:17769Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. spermidine:(acceptor) oxidoreductase

  Catalysed by NAGS, SAT1, SAT2mitochondrial matrix, cytosol

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

  Catalysed by CTH, SLC7A6, HALcytosol, extracellular space

• 5-hydroxy-L-tryptophanReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  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 CTH, SLC7A5, TPH1, TPH2cytosol, extracellular space

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

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

• a triacylglycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 6 moremitochondrial matrix, Golgi apparatus, cytosol

• 4-methyl-2-oxopentanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine

  Catalysed by CTH, AGXT2, AADATcytosolOne of 5 routes

• butanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + H2O = butanoate + CoA + H(+)

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 9 moremitochondrial matrix, cytosol

• 3-phenylpyruvateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine

  Catalysed by CTH, AGXT2, AADATcytosolOne of 6 routes

• thiocyanateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-mercaptopyruvate sulfurtransferase (hydrogen-cyanide)

  Catalysed by CTH, GOT2, GOT1, MPST and 1 morecytosol

• dTDPReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. pyruvate kinase (dTDP)

  Catalysed by CTH, AGXT2, PKM, PKLRcytosol

• spermine monoaldehydeReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. diamine N-acetyltransferase (N1-acetylspermine)
  3. primary-amine oxidase (spermine)

  Catalysed by NAGS, SAT1, SAT2, AOC2 and 2 moremitochondrial matrix, cytosolOne of 3 routes

• CHEBI:18095Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. Transport of Hydroxy-Proline

  Catalysed by CTH, SLC7A5, SLC1A4cytosol, extracellular spaceOne of 5 routes

• O-octanoyl-(R)-carnitineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Carnitine O-Palmitoyltransferase
  3. solute carrier (L-carnitine)

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 1 moremitochondrial matrix, cytosolOne of 11 routes

• CHEBI:18132Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Acetyl-CoA:choline O-acetyltransferase
  3. ATP:choline phosphotransferase

  Catalysed by NAGS, CHAT, CHKA, CHKBmitochondrial matrix, nucleus, cytosol

• CHEBI:18183Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-glutamate:L-cysteine gamma-ligase (ADP-forming)
  3. gamma-glutamylcyclotransferase (gamma-glutamyl-cysteine)

  Catalysed by CTH, GCLC, GCLM, GGCT and 1 morecytosol

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

  Catalysed by CTH, SLC6A14cytosol, extracellular spaceOne of 2 routes

• dodecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. Lauroyl-CoA hydrolase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular space

• N-(4-aminobutyl)-3-aminopropanalReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. primary-amine oxidase (spermidine)

  Catalysed by NAGS, SAT1, SAT2, AOC2 and 2 moremitochondrial matrix, cytosolOne of 3 routes

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

  Catalysed by CTH, SLC7A5, DDCcytosol, extracellular space

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

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A10 and 1 morecytosol, extracellular space

• (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (EPA)
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + O2 = (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoA + H2O2

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

• gamma-L-glutamyl-(2S)-2-aminobutanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O
  3. (2S)-2-aminobutanoate + L-glutamate + ATP = gamma-L-glutamyl-(2S)-2-aminobutanoate + ADP + phosphate + H(+)

  Catalysed by CTH, AADAT, GCLCcytosol

• 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. a 2-acyl-sn-glycero-3-phosphoethanolamine + octadecanoyl-CoA = 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosol

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

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular space

• S-methylmercury-L-cysteineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  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 CTH, SLC7A5, SLC7A8cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 6 routes

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

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

• 11-cis-retinyl esterReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol + a fatty acyl-CoA = 11-cis-retinyl ester + CoA

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

• (6R)-10-formyltetrahydrofolateReach 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. (6S)-5,6,7,8-tetrahydrofolate + formate + ATP = (6R)-10-formyltetrahydrofolate + ADP + phosphate

  Catalysed by CBS, MTR, MTHFD1, MTHFD1L

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  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 CTH, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular spaceOne of 2 routes

• 5-(3,3'-dimethylguanidino)-2-oxopentanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by CTH, AGXT2cytosolOne of 2 routes

• 5-(3-methylguanidino)-2-oxopentanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. N(omega)-methyl-L-arginine + glyoxylate = 5-(3-methylguanidino)-2-oxopentanoate + glycine

  Catalysed by CTH, AGXT2cytosolOne of 2 routes

• 1-butanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Butanoate:CoA ligase (AMP-forming)
  3. 2-(9Z-octadecenoyl)-glycerol + butanoyl-CoA = 1-butanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 5 moremitochondrial matrix, cytosol

• 1-octanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. 2-(9Z-octadecenoyl)-glycerol + octanoyl-CoA = 1-octanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by NAGS, CROT, MOGAT2, MOGAT1mitochondrial matrix

• 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + 2-(9Z-octadecenoyl)-glycerol = 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

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

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

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular spaceOne of 2 routes

• O-butanoyl-(R)-carnitineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + (R)-carnitine = O-butanoyl-(R)-carnitine + CoA

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 4 moremitochondrial matrix, cytosol

• 3-oxo-(9Z)-eicosenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)
  3. very-long-chain 3-oxoacyl-CoA synthase ((7Z)-octadecenoyl-CoA)

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 7 moremitochondrial matrix, cytosol

• (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (gamma-linolenate)
  3. (6Z,9Z,12Z)-octadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 8 moremitochondrial matrix, cytosolOne of 2 routes

• (2E,7Z,10Z)-hexadecatrienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial
  3. (7Z,10Z)-hexadecadienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z)-hexadecatrienoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by NAGS, HADHA, ACADVL, HADHB and 2 moremitochondrial matrix

• (2E,4Z,7Z,10Z,13Z)-hexadecapentaenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial
  3. Fatty Acid Beta Oxidation (C16:4->C16:5), Mitochondrial

  Catalysed by NAGS, HADHA, ACADVL, HADHBmitochondrial matrixOne of 4 routes

• (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial
  3. (7Z,10Z,13Z)-hexadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by NAGS, HADHA, ACADVL, HADHB and 2 moremitochondrial matrix

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

  Catalysed by NAGS, ECHS1, ACAA2, HADH and 1 moremitochondrial matrixOne of 3 routes

• docosanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. Docosanoyl-CoA hydrolase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosolOne of 4 routes

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

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 3 morecytosol, extracellular space, mitochondrial matrixOne of 4 routes

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

  Catalysed by CTH, SQORcytosol

• hypochlorous acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. Peroxidase

  Catalysed by CTH, SLC6A14, LPOcytosol, extracellular spaceOne of 4 routes

• octadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. Stearoyl-CoA hydrolase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosolOne of 4 routes

• octanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. Octanoyl-CoA hydrolase

  Catalysed by NAGS, CROT, ACOT7mitochondrial matrix, cytosolOne of 2 routes