L,L-cystathionine

Three steps

• (2E,4Z,7Z,10Z,13Z,16Z,19Z)-docosaheptaenoyl-CoAReach 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 + O2 = (2E,4Z,7Z,10Z,13Z,16Z,19Z)-docosaheptaenoyl-CoA + H2O2

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

• N-(9Z-octadecenoyl)-sphing-4-enineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. N-(9Z-octadecenoyl)-sphing-4-enine + H2O = sphing-4-enine + (9Z)-octadecenoate

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 2 moremitochondrial matrix, Golgi apparatus

• 9-cis-retinolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol = 9-cis-retinol

  Catalysed by NAGS, DGAT1, LRAT, AWAT1 and 3 moremitochondrial matrix, cytosol

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

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

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

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

• prostaglandin G2Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin G2 (cytosol to extracellular)

  Catalysed by CTH, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• cholesteryl octadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

• N-octadecanoylhexadecasphinganineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. hexadecasphinganine + octadecanoyl-CoA = N-octadecanoylhexadecasphinganine + CoA + H(+)

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

• 1-hexadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphateReach 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 + 1-hexadecanoyl-sn-glycero-3-phosphate = 1-hexadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphate + CoA

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

• 1-hexadecanoyl-2-eicosanoyl-sn-glycero-3-phosphocholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + 1-hexadecanoyl-sn-glycero-3-phosphocholine = 1-hexadecanoyl-2-eicosanoyl-sn-glycero-3-phosphocholine + CoA

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

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

  Catalysed by CTH, SLC3A2, SLC7A5, SLC7A8cytosol, extracellular spaceOne of 3 routes

• sphinga-4E,14Z-dienineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = sphinga-4E,14Z-dienine + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by NAGS, ASAH1, ACER2, ACER1 and 2 moremitochondrial matrix, Golgi apparatus

• 1',3'-bis-[1,2-di-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. 1'-[1,2-di-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-3'-[1-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-glycerol + (9Z,12Z)-octadecadienoyl-CoA = 1',3'-bis-[1,2-di-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phospho]-glycerol + CoA

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

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

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

• cholesteryl (9Z)-hexadecenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (palmitolate)
  3. (9Z)-hexadecenoyl-CoA + cholesterol = cholesteryl (9Z)-hexadecenoate + CoA

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

• (9Z,12Z,15Z-octadecatrienoyl)-cholesterolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (linolenate)
  3. (9Z,12Z,15Z)-octadecatrienoyl-CoA + cholesterol = (9Z,12Z,15Z-octadecatrienoyl)-cholesterol + CoA

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

• cholesteryl (8Z,11Z,14Z)-eicosatrienoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)
  3. Acyl-CoA:cholesterol O-acyltransferase

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

• O-tetradecanoyl-(R)-carnitineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. tetradecanoyl-CoA + (R)-carnitine = O-tetradecanoyl-(R)-carnitine + CoA

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

• O-octadecanoyl-(R)-carnitineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. (R)-carnitine + octadecanoyl-CoA = O-octadecanoyl-(R)-carnitine + CoA

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

• O-eicosanoyl-(R)-carnitineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + (R)-carnitine = O-eicosanoyl-(R)-carnitine + CoA

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

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

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 2 moremitochondrial matrix, cytosol, peroxisomeOne of 7 routes

• 1-octadecanoyl-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 (stearate)
  3. 2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphocholine + octadecanoyl-CoA = 1-octadecanoyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholine + CoA

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

• 1-octadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. 2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholine + octadecanoyl-CoA = 1-octadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholine + CoA

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

• (5Z,8Z,11Z,14Z,17Z-eicosapentaenoyl)-cholesterolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (EPA)
  3. Acyl-CoA:cholesterol O-acyltransferase

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol, endoplasmic reticulumOne of 2 routes

• N-tetradecanoylglycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. tetradecanoyl-CoA + glycine = N-tetradecanoylglycine + CoA + H(+)

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

• N-eicosanoylglycinateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. eicosanoyl-CoA + glycine = N-eicosanoylglycinate + CoA + H(+)

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

• N-docosanoylglycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. docosanoyl-CoA + glycine = N-docosanoylglycine + CoA + H(+)

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

• N-hexacosanoylglycineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. hexacosanoyl-CoA + glycine = N-hexacosanoylglycine + CoA + H(+)

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

• an N(2)-acyl-L-glutamineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)

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

• (2E,5Z)-tetradecadienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (physeteric acid)
  3. medium-chain acyl-CoA dehydrogenase (5-tetradecenoyl-CoA)

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

• (2E,7Z)-hexadecadienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)
  3. medium-chain acyl-CoA dehydrogenase (7-hexadecenoyl-CoA)

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

• 3-methylmalateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)

  Catalysed by CTH, AGXT2, CLYBLcytosol

• cholesteryl (11Z)-octadecenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix
  3. (11Z)-octadecenoyl-CoA + cholesterol = cholesteryl (11Z)-octadecenoate + CoA

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 1 moremitochondrial matrix, cytosol

• Se-methylselenocysteineReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. Selenocystathionine Lysteine-lyase (deaminating)
  3. L-Serine hydro-lyase (adding homocysteine)

  Catalysed by CTH, CBScytosol

• thromboxane B2Reach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. transport of GSH and thromboxane B2 (cytosol to extracellular)

  Catalysed by CTH, SLCO1B1cytosol, extracellular spaceOne of 2 routes

• a 3-oxoacyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + acetyl-CoA = a 3-oxoacyl-CoA + CoA

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

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

  Catalysed by CTH, SLCO1A2, SLC7A9, SLCO1B3cytosol, extracellular spaceOne of 7 routes

• thiamine(1+) diphosphateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)
  3. lipoamide to thiamin-PP conversion

  Catalysed by CTH, BCKDHA, BCKDHB, DBTcytosol, mitochondrial matrix

• 2-(1-hydroxyethyl)thiamine diphosphateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. pyruvate:thiamin diphosphate acetaldehydetransferase (decarboxylating)

  Catalysed by CTH, AGXT2, PDHA1, PDHB and 1 morecytosol, mitochondrial matrix