L,L-cystathionine

Two steps

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

  Catalysed by CTH, SLC7A5cytosol, extracellular spaceOne of 6 routes

• gamma-L-glutamyl-L-cysteineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-glutamate:L-cysteine gamma-ligase (ADP-forming)

  Catalysed by CTH, GCLC, GCLMcytosolOne of 2 routes

• N,N-dimethylglycineReach from here →
  1. L-homocysteine + L-serine = L,L-cystathionine + H2O
  2. L-homocysteine + glycine betaine = N,N-dimethylglycine + L-methionine

  Catalysed by CBS, BHMT

• an acyl-CoAReach 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

• kynurenateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O

  Catalysed by CTH, AADATcytosol

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

  Catalysed by NAGS, HADHA, ACAA2, HADHBmitochondrial matrixOne of 3 routes

• S-sulfanyl-L-cysteineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2 L-cysteine = S-sulfanyl-L-cysteine + L-alanine

  Catalysed by CTH, CARS2cytosol

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by NAGS, SLC27A2, SLC27A5mitochondrial matrix, endoplasmic reticulumOne of 3 routes

• deoxycholoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by NAGS, SLC27A2, SLC27A5mitochondrial matrix, cytosolOne of 4 routes

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by CTH, PPCScytosolOne of 4 routes

• 3-sulfino-L-alanineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. L-Cysteine:oxygen oxidoreductase

  Catalysed by CTH, CDO1cytosolOne of 2 routes

• decanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 3 moremitochondrial matrix, cytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Carnitine O-Palmitoyltransferase

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (palmitolate)

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

• 3-hydroxyisovaleryl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by NAGS, CPT1A, CPT1Bmitochondrial matrix, cytosol

• CHEBI:63544Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (adrenic acid)

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

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

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

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 10 routes

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

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

• (2E)-octadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Carnitine O-Palmitoyltransferase

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearidonic acid)

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

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

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

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (linolenate)

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

• (13Z)-docosenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

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

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

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

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

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

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

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

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

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

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

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

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

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

• (2S)-2-aminobutanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O

  Catalysed by CTH, AADATcytosolOne of 5 routes

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Activation of dodecanedioic acid

  Catalysed by NAGSmitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Production of Sebacoylcarnitine

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Production of Suberylcarnitine

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by NAGSmitochondrial matrix, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

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

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

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

• 2-iminobutanoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-iminobutanoate + H2O = 2-oxobutanoate + NH4(+)

  Catalysed by CTH, RIDAcytosol

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

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

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by NAGS, CRAT, CROTmitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by NAGS, ACAA1, HSD17B4, EHHADH and 1 moremitochondrial matrix, peroxisome