L,L-cystathionine

Three steps

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

  Catalysed by CTH, SLC6A14, SLC12A2, SLC12A1cytosol, extracellular spaceOne of 2 routes

• alpha-linolenic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

• phenylacetylglycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular spaceOne of 6 routes

• decanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. Decanoyl-CoA hydrolase

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 4 moremitochondrial matrix, cytosolOne of 2 routes

• all-cis-docosa-4,7,10,13,16,19-hexaenoic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (DHA)
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA hydrolase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosolOne of 2 routes

• 5-hydroxytryptophanReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-Tryptophan, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)

  Catalysed by CTH, SLC7A5, TPH1, TPH2cytosol, extracellular spaceOne of 2 routes

• 5(S)-HETEReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. Glutathione: 5-HPETE oxidoreductase

  Catalysed by CTH, GPX5, GPX1, GPX2 and 6 morecytosolOne of 2 routes

• hexacosan-1-olReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + 2 NADPH + 2 H(+) = hexacosan-1-ol + 2 NADP(+) + CoA

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

• 3-mercaptolactic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-Mercaptolactate:NAD+ oxidoreductase

  Catalysed by CTH, GOT2, GOT1, LDHB and 3 morecytosol

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

  Catalysed by CTH, AGXT2cytosol

• CHEBI:28666Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. LTD4 hydrolysis

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8cytosol, extracellular spaceOne of 4 routes

• O-decanoyl-(R)-carnitineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. decanoyl-CoA + (R)-carnitine = O-decanoyl-(R)-carnitine + CoA

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

• pyridoxine 5'-phosphateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of alanine and cysteine (cytosol to extracellular)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by CTH, SLC7A5, SCLYcytosol, extracellular space

• 3-disulfanyl-L-alanineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. thioredoxin-disulfide reductase (NADPH) (cystine)
  3. L-cystine thiocysteine-lyase (deaminating

  Catalysed by CTH, TXNRD1, TXNRD3, TXNRD2cytosol

• a dicarboxylateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O
  3. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)

  Catalysed by CTH, AADAT, SLC22A8cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• Ca(2+)Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)
  3. Ca(2+)(out) + K(+)(out) + 4 Na(+)(in) = Ca(2+)(in) + K(+)(in) + 4 Na(+)(out)

  Catalysed by CTH, SLC1A1, SLC24A1, SLC24A5 and 3 morecytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• Phe-tRNA(Phe)Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-Phenylalanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by CTH, SLC7A5, FARSB, FARSAcytosol, extracellular space

• Gly-tRNA(Gly)Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. glycine:tRNA(Gly) ligase (AMP-forming)

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

• Trp-tRNA(Trp)Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-Tryptophan -tRNA(Trp) ligase (AMP-forming)

  Catalysed by CTH, SLC7A5, WARS1cytosol, extracellular space

• Ile-tRNA(Ile)Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and isoleucine (cytosol to extracellular)
  3. L-Isoleucine:tRNA(Ile) ligase (AMP-forming)

  Catalysed by CTH, SLC7A5, IARS1cytosol, extracellular space

• Gln-tRNA(Gln)Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. L-Glutamine:tRNA(Gln) ligase (AMP-forming)

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

• isocyanateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. L-citrulline + H(+) = isocyanate + L-ornithine

  Catalysed by CTH, SLC7A6, LACC1cytosol, extracellular space

• a thiolReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. L-cysteinyl-[sulfatase] + 2 a thiol + O2 = an organic disulfide + 3-oxo-L-alanyl-[sulfatase] + hydrogen sulfide + H2O + H(+)

  Catalysed by CTH, SUMF1cytosol

• Asn-tRNA(Asn)Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of asparagine and cysteine (cytosol to extracellular)
  3. L-Asparagine:tRNA(Asn) ligase (AMP-forming)

  Catalysed by CTH, SLC7A5, NARS1cytosol, extracellular space

• phosphocholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Acetyl-CoA:choline O-acetyltransferase
  3. phosphocholine + H2O = choline + phosphate

  Catalysed by NAGS, CHAT, PHOSPHO1mitochondrial matrix, nucleusOne of 2 routes

• glycocholateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8 and 1 morecytosol, extracellular space, peroxisomeOne of 7 routes

• cholateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. solute carrier organic anion transporter (cholate)

  Catalysed by CTH, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 4 routes

• glycolateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. glycolate + NADP(+) = glyoxylate + NADPH + H(+)

  Catalysed by CTH, AGXT2, GRHPRcytosol

• D-aspartateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)
  3. D-aspartate(out) + K(+)(in) + 3 Na(+)(out) + H(+)(out) = D-aspartate(in) + K(+)(out) + 3 Na(+)(in) + H(+)(in)

  Catalysed by CTH, SLC1A1, SLC1A7, SLC1A3 and 2 morecytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-aspartateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-Cysteine:oxygen oxidoreductase
  3. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)

  Catalysed by CTH, CDO1, SLC25A12, SLC25A13cytosol, mitochondrial matrixOne of 31 routes

• acetateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-hydroxybutyrate:NAD+ oxidoreductase
  3. transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)

  Catalysed by CTH, LDHB, LDHC, LDHAL6A and 5 morecytosol, extracellular spaceOne of 4 routes

• (9Z,12Z)-octadecadienoateReach 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 + H2O = (9Z,12Z)-octadecadienoate + CoA + H(+)

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

• CHEBI:30249Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)

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

• 3-acetamidopropanalReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. N1,N12-diacetylspermine to N1-acetylspermine conversion
  3. N1-acetylspermine:oxygen oxidoreductase (3-acetamidopropanal-forming)

  Catalysed by NAGS, PAOX, SMOXmitochondrial matrix, cytosolOne of 4 routes

• oxalateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. glyoxylate + O2 + H2O = oxalate + H2O2 + H(+)

  Catalysed by CTH, AGXT2, HAO1cytosol

• formic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-hydroxybutyrate:NAD+ oxidoreductase
  3. transport of 2-hydroxybutyrate and formate (cytosol to extracellular)

  Catalysed by CTH, LDHB, LDHC, LDHAL6A and 5 morecytosol, extracellular spaceOne of 6 routes

• propionic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-hydroxybutyrate:NAD+ oxidoreductase
  3. transport of 2-hydroxybutyrate and propanoate (cytosol to extracellular)

  Catalysed by CTH, LDHB, LDHC, LDHAL6A and 5 morecytosol, extracellular spaceOne of 7 routes

• citric acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. ATP citrate lyase (citrate)
  3. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)

  Catalysed by NAGS, ACLY, CSmitochondrial matrix, cytosolOne of 2 routes

• butyric acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-hydroxybutyrate:NAD+ oxidoreductase
  3. transport of 2-hydroxybutyrate and butyrate (cytosol to extracellular)

  Catalysed by CTH, LDHB, LDHC, LDHAL6A and 5 morecytosol, extracellular spaceOne of 5 routes

• malonic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. Malonate Transport, Antiport with Chloride

  Catalysed by CTH, SLC6A14cytosol, extracellular spaceOne of 2 routes

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 10 moremitochondrial matrix, cytosolOne of 2 routes

• (9Z)-octadecenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol, endoplasmic reticulumOne of 3 routes

• adipic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Activation of adipic acid for Formation of Adipoyl Carnitine
  3. Thioesterification of adipoyl-CoA for Release into Cytosol

  Catalysed by NAGS, ACOT8mitochondrial matrix, cytosol, peroxisomeOne of 3 routes

• 3-hydroxypyruvic acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. L-Serine:pyruvate aminotransferase

  Catalysed by CTH, AGXT2, AGXTcytosolOne of 2 routes

• glutarateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O
  3. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)

  Catalysed by CTH, AADAT, SLC22A6cytosol, inner side of the membrane, outer side of the membraneOne of 7 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. ATP citrate lyase (citrate)
  3. D-aspartate:2-oxoglutarate aminotransferase

  Catalysed by NAGS, ACLY, GOT2mitochondrial matrix, cytosol

• docosan-1-olReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. docosanoyl-CoA + 2 NADPH + 2 H(+) = docosan-1-ol + 2 NADP(+) + CoA

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

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosolOne of 2 routes

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

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

• an N-acylsphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphingosine N-acyltransferase (sphinganine)

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