L,L-cystathionine

Two steps

• (5Z)-dodecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by NAGS, HADHA, ACAA2, HADHBmitochondrial matrixOne of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• (7Z)-tetradecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 8 routes

• L-homocysteine thiolactoneReach from here →
  1. L-homocysteine + L-serine = L,L-cystathionine + H2O
  2. L-homocysteine + ATP = L-homocysteine thiolactone + AMP + diphosphate

  Catalysed by CBS, MARS1

• L-homocysteinyl-AMPReach from here →
  1. L-homocysteine + L-serine = L,L-cystathionine + H2O
  2. L-homocysteine + ATP + H(+) = L-homocysteinyl-AMP + diphosphate

  Catalysed by CBS, MARS1

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

  Catalysed by NAGS, HADHA, ACADVL, HADHBmitochondrial matrixOne of 3 routes

• (7Z,10Z)-hexadecadienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by NAGS, HADHA, ACADVL, HADHBmitochondrial matrix

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

  Catalysed by NAGS, ECHS1, ACAA2, HADHmitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by NAGS, ECHS1, ACAA2, HADHmitochondrial matrixOne of 2 routes

• CHEBI:27540Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 6 routes

• selenomethionineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. Selenomethionine methanethiol-lyase (deaminating)

  Catalysed by CTHcytosol

• CHEBI:27760Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. Selenocystathionine Lysteine-lyase (deaminating)

  Catalysed by CTHcytosol

• elaidic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (elaidate)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• CHEBI:28701Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by NAGS, ACAA1, SCP2, MYO5Bmitochondrial matrix, peroxisomeOne of 7 routes

• N-acetyl-L-cysteineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)

  Catalysed by CTH, NAGScytosol, mitochondrial matrix

• K(+)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)

  Catalysed by CTH, SLC1A1cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• Cys-tRNA(Cys)Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-Cysteine:tRNA(Cys) ligase (AMP-forming)

  Catalysed by CTH, CARS1cytosol

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

  Catalysed by CTH, SLC6A14cytosol, extracellular spaceOne of 2 routes

• hydrogen sulfideReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-cysteine hydrogen-sulfide-lyase (deaminating

  Catalysed by CTHcytosolOne of 3 routes

• di-mu-sulfido-diiron(2+)Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. De novo synthesis of 2Fe2S

  Catalysed by CTH, NDUFAB1, FDXR, FXN and 4 morecytosol, mitochondrial matrix

• L-alpha-aminobutyric acidReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. glutamic-pyruvic transaminase ((S)-2-aminobutanoate)

  Catalysed by CTH, GPTcytosol

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

  Catalysed by CTH, AGXT2cytosol

• L-ornithineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and ornithine (cytosol to extracellular)

  Catalysed by CTH, SLC7A6cytosol, extracellular spaceOne of 2 routes

• taurineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by NAGS, BAATmitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8cytosol, extracellular spaceOne of 14 routes

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

  Catalysed by NAGS, ALDH6A1mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. (S)-methylmalonyl-CoA hydrolase

  Catalysed by NAGSmitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acid phosphatase 2, lysosomal (CoA)

  Catalysed by NAGS, ACP2mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. (2E)-dodecenoyl-CoA formation

  Catalysed by NAGSmitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by NAGS, ACAA1, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• butanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Butanoate:CoA ligase (AMP-forming)

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 9 routes

• dodecanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 9 routes

• glutaryl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Production of Glutaryl Carnitine

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

• eicosanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 12 routes

• itaconyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Itaconate:CoA ligase (GDP-forming)

  Catalysed by NAGS, SUCLG1, SUCLG2, SUCLA2mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 13 routes

• tetradecanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 11 routes

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

  Catalysed by NAGS, CROTmitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. phytanate-CoA ligase

  Catalysed by NAGSmitochondrial matrix, cytosolOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 4 moremitochondrial matrix, cytosolOne of 15 routes

• (6S)-5,6,7,8-tetrahydrofolateReach 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

  Catalysed by CBS, MTR

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-Cysteine:2-oxoglutarate aminotransferase

  Catalysed by CTH, GOT2, GOT1cytosolOne of 3 routes

• sphing-4-enineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 1 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• spermidineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. diamine N-acetyltransferase (N1-acetylspermidine)

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

• L-methionineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and methionine (cytosol to extracellular)

  Catalysed by CTH, SLC7A5cytosol, extracellular spaceOne of 10 routes

• L-tryptophanReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and tryptophan (cytosol to extracellular)

  Catalysed by CTH, SLC7A5cytosol, extracellular spaceOne of 4 routes

• L-threonineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-threonine = 2-oxobutanoate + NH4(+)

  Catalysed by CTH, SDS, SDSLcytosol

• L-alanineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of alanine and cysteine (cytosol to extracellular)

  Catalysed by CTH, SLC7A5cytosol, extracellular spaceOne of 15 routes

• L-asparagineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of asparagine and cysteine (cytosol to extracellular)

  Catalysed by CTH, SLC7A5cytosol, extracellular spaceOne of 5 routes