L,L-cystathionine

Three steps

• L-dopaquinoneReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,L-dopa:oxygen oxidoreductase

  Catalysed by CTH, SLC7A5, TYRcytosol, extracellular space

• glutathioneReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by CTH, TSTD1cytosolOne of 3 routes

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

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

• L-kynurenineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

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

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

  Catalysed by CTH, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 5 routes

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

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 5 moremitochondrial matrix, Golgi apparatus

• leukotriene C4Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. leukotriene C4 hydrolysis

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8cytosol, extracellular spaceOne of 5 routes

• taurocyamineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by CTH, SLC7A6, GATMcytosol, extracellular spaceOne of 2 routes

• L-glutamate 5-semialdehydeReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate

  Catalysed by CTH, SLC7A6, OATcytosol, extracellular space

• L-cysteateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)
  3. L-cysteate + H(+) = taurine + CO2

  Catalysed by NAGS, BAAT, GADL1, CSADmitochondrial matrix, cytosol

• N(1)-acetylspermineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. N(1)-acetylspermine + O2 + H2O = 3-acetamidopropanal + spermidine + H2O2

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

• 3-hydroxy-L-kynurenineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by CTH, SLC7A5, KYNUcytosol, extracellular space

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

  Catalysed by CTH, SLC7A6cytosol, extracellular space, peroxisome

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

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A10 and 1 morecytosol, extracellular spaceOne of 4 routes

• S-methyl-L-methionineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by CTH, SLC7A5, BHMT2cytosol, extracellular space

• glutathione disulfideReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. thioredoxin-disulfide reductase (NADPH) (cystine)
  3. cystine to cysteine conversion

  Catalysed by CTH, TXNRD1, TXNRD3, TXNRD2cytosolOne of 14 routes

• 3-oxosphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. Palmitoyl-CoA:L-serine C-palmitoyltransferase (decarboxylating)

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

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

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

• 1-hexadecanoylglycerone 3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Palmitate:CoA ligase (AMP-forming)
  3. glyceronephosphate O-acyltransferase (DHAP)

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

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

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

• N(1)-acetylspermidineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. spermidine + acetyl-CoA = N(1)-acetylspermidine + CoA + H(+)

  Catalysed by NAGS, SAT1, SAT2mitochondrial matrix, cytosol

• N(omega),N(omega)-dimethyl-L-arginineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O
  3. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate

  Catalysed by CTH, AADAT, AGXT2cytosolOne of 13 routes

• 3-aminopropanalReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. diamine N-acetyltransferase (N1-acetylspermine)
  3. spermidine:oxygen oxidoreductase (spermidine-forming)

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

• trans-4-hydroxy-L-prolineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-threonine = 2-oxobutanoate + NH4(+)
  3. trans-4-hydroxy-L-proline(out) + L-threonine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-threonine(out) + Na(+)(in)

  Catalysed by CTH, SDS, SDSL, SLC1A4cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

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

  Catalysed by CTH, SLC7A6, HDCcytosol, extracellular spaceOne of 3 routes

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

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

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

  Catalysed by CTH, SLC7A6, IL4I1cytosol, extracellular space

• N(8)-acetylspermidineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. N8-acetylspermidine to spermidine conversion

  Catalysed by NAGS, SAT1, SAT2mitochondrial matrix, cytosolOne of 3 routes

• N(1),N(12)-diacetylspermineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. N1,N12-diacetylspermine to N1-acetylspermine conversion
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by NAGSmitochondrial matrix, cytosolOne of 2 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoateReach 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 hydrolase

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

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

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 14 moremitochondrial matrix, Golgi apparatus

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

  Catalysed by CTH, SLC7A5, IDO1, TDO2 and 1 morecytosol, extracellular space

• (3S)-citramalyl-CoAReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by CTH, AGXT2, CLYBLcytosolOne of 2 routes

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

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A10 and 1 morecytosol, extracellular spaceOne of 2 routes

• phosphoenolpyruvateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. ATP citrate lyase (citrate)
  3. oxaloacetate + GTP = phosphoenolpyruvate + GDP + CO2

  Catalysed by NAGS, ACLY, PCK1, PCK2mitochondrial matrix, cytosol

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

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A10 and 2 morecytosol, extracellular space

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

  Catalysed by CTH, SLC7A5, MSRAcytosol, extracellular space

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

  Catalysed by CTH, SLC7A5, MSRB3, MSRB1 and 1 morecytosol, extracellular space

• 5-amino-2-oxopentanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. L-ornithine + glyoxylate = 5-amino-2-oxopentanoate + glycine

  Catalysed by CTH, AGXT2cytosolOne of 4 routes

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

  Catalysed by CTH, SQORcytosol, mitochondrial matrixOne of 4 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  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 CTH, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular spaceOne of 2 routes

• 4-aminobutanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by CTH, SLC6A14, SLC6A12cytosol, extracellular spaceOne of 4 routes

• 3-iodo-L-tyrosineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

  Catalysed by CTH, SLC7A5, TPOcytosol, extracellular spaceOne of 2 routes

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

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

• 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. (9Z,12Z)-octadecadienoyl-CoA + a 1-acyl-sn-glycero-3-phosphocholine = 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholine + CoA

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

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

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

• a 2,3-dehydroacyl-CoAReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  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 CTH, MOCOS, ACAD10cytosol

• L-prolineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  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 CTH, SLC7A5, SLC1A4cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• sphing-4-enine 1-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + ATP = sphing-4-enine 1-phosphate + ADP + H(+)

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

• prostaglandin E2Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin E2 (cytosol to extracellular)

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