L,L-cystathionine

Three steps

• 1-(9Z)-octadecenoyl-2-dodecanoyl-sn-glycero-3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. 1-(9Z-octadecenoyl)-sn-glycero-3-phosphate + dodecanoyl-CoA = 1-(9Z)-octadecenoyl-2-dodecanoyl-sn-glycero-3-phosphate + CoA

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

• 1-(9Z)-octadecenoyl-2-nonadecanoyl-sn-glycero-3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (nonadecylic acid)
  3. nonadecanoyl-CoA + 1-(9Z-octadecenoyl)-sn-glycero-3-phosphate = 1-(9Z)-octadecenoyl-2-nonadecanoyl-sn-glycero-3-phosphate + CoA

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

• 1-(9Z)-octadecenoyl-2-(11Z)-octadecenoyl-sn-glycero-3-phosphateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix
  3. (11Z)-octadecenoyl-CoA + 1-(9Z-octadecenoyl)-sn-glycero-3-phosphate = 1-(9Z)-octadecenoyl-2-(11Z)-octadecenoyl-sn-glycero-3-phosphate + CoA

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 2 moremitochondrial matrix, cytosol

• 1-hexadecanoyl-2-dodecanoyl-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 (lauric acid)
  3. 1-hexadecanoyl-sn-glycero-3-phospho-(1D-myo-inositol) + dodecanoyl-CoA = 1-hexadecanoyl-2-dodecanoyl-sn-glycero-3-phospho-(1D-myo-inositol) + CoA

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

• 1'-[1-acyl-2-dodecanoyl-sn-glycero-3-phospho],3'-[1-acyl,2-hydroxy-sn-glycero-3-phospho]-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. 1',3'-bis-[1-acyl-sn-glycero-3-phospho]-glycerol + dodecanoyl-CoA = 1'-[1-acyl-2-dodecanoyl-sn-glycero-3-phospho],3'-[1-acyl,2-hydroxy-sn-glycero-3-phospho]-glycerol + CoA

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

• 1'-[1,2-diacyl-sn-glycero-3-phospho],3'-[1-acyl,2-dodecanoyl-sn-glycero-3-phospho]-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. 1'-[1,2-diacyl-sn-glycero-3-phospho],3'-[1-acyl-sn-glycero-3-phospho]-glycerol + dodecanoyl-CoA = 1'-[1,2-diacyl-sn-glycero-3-phospho],3'-[1-acyl,2-dodecanoyl-sn-glycero-3-phospho]-glycerol + CoA

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

• 1'-[1,2-diacyl-sn-glycero-3-phospho],3'-[1-acyl,2-(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-diacyl-sn-glycero-3-phospho],3'-[1-acyl-sn-glycero-3-phospho]-glycerol + (9Z,12Z)-octadecadienoyl-CoA = 1'-[1,2-diacyl-sn-glycero-3-phospho],3'-[1-acyl,2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phospho]-glycerol + CoA

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

• 1'-[1-acyl-2-octadecanoyl-sn-glycero-3-phospho],3'-[1-acyl,2-hydroxy-sn-glycero-3-phospho]-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. 1',3'-bis-[1-acyl-sn-glycero-3-phospho]-glycerol + octadecanoyl-CoA = 1'-[1-acyl-2-octadecanoyl-sn-glycero-3-phospho],3'-[1-acyl,2-hydroxy-sn-glycero-3-phospho]-glycerol + CoA

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

• 1'-[1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phospho],3'-[1-acyl,2-hydroxy-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',3'-bis-[1-acyl-sn-glycero-3-phospho]-glycerol + (9Z,12Z)-octadecadienoyl-CoA = 1'-[1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phospho],3'-[1-acyl,2-hydroxy-sn-glycero-3-phospho]-glycerol + CoA

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

• 1-acyl-2-octanoyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. octanoyl-CoA + a 1-acyl-sn-glycero-3-phosphoethanolamine = 1-acyl-2-octanoyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by NAGS, CROT, LPCAT4mitochondrial matrix

• 1-acyl-2-octadecanoyl-sn-glycero-3-phosphoethanolamineReach 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-phosphoethanolamine + octadecanoyl-CoA = 1-acyl-2-octadecanoyl-sn-glycero-3-phosphoethanolamine + CoA

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

• 1-hexadecanoyl-2-decanoyl-sn-glycero-3-phosphocholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. decanoyl-CoA + 1-hexadecanoyl-sn-glycero-3-phosphocholine = 1-hexadecanoyl-2-decanoyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by NAGS, ACSM4, ACSM1, ACSM2A and 4 moremitochondrial matrix, cytosol

• 1-hexadecanoyl-2-octanoyl-sn-glycero-3-phosphocholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. octanoyl-CoA + 1-hexadecanoyl-sn-glycero-3-phosphocholine = 1-hexadecanoyl-2-octanoyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by NAGS, CROT, LPCAT1mitochondrial matrix

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

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

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

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

• 1-dodecanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. 2-(9Z-octadecenoyl)-glycerol + dodecanoyl-CoA = 1-dodecanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

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

• 1-tetradecanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (myristic acid)
  3. 2-(9Z-octadecenoyl)-glycerol + tetradecanoyl-CoA = 1-tetradecanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

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

• 1-octadecanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. 2-(9Z-octadecenoyl)-glycerol + octadecanoyl-CoA = 1-octadecanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

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

• 1-(9Z,12Z-octadecadienoyl)-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)
  3. 2-(9Z-octadecenoyl)-glycerol + (9Z,12Z)-octadecadienoyl-CoA = 1-(9Z,12Z-octadecadienoyl)-2-(9Z-octadecenoyl)-glycerol + CoA

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

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

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

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

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

• hexadecyl octadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. hexadecan-1-ol + octadecanoyl-CoA = hexadecyl octadecanoate + CoA

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

• 1'-[1,2-diacyl-sn-glycero-3-phospho],3'-[1-acyl,2-octanoyl-sn-glycero-3-phospho]-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. 1'-[1,2-diacyl-sn-glycero-3-phospho],3'-[1-acyl-sn-glycero-3-phospho]-glycerol + octanoyl-CoA = 1'-[1,2-diacyl-sn-glycero-3-phospho],3'-[1-acyl,2-octanoyl-sn-glycero-3-phospho]-glycerol + CoA

  Catalysed by NAGS, CROT, LCLAT1mitochondrial matrix

• 1'-[1-acyl-2-octanoyl-sn-glycero-3-phospho],3'-[1-acyl,2-hydroxy-sn-glycero-3-phospho]-glycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. 1',3'-bis-[1-acyl-sn-glycero-3-phospho]-glycerol + octanoyl-CoA = 1'-[1-acyl-2-octanoyl-sn-glycero-3-phospho],3'-[1-acyl,2-hydroxy-sn-glycero-3-phospho]-glycerol + CoA

  Catalysed by NAGS, CROT, LCLAT1mitochondrial matrix

• 1-O-alkyl-3-acylglycerolReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-O-alkylglycerol + an acyl-CoA = 1-O-alkyl-3-acylglycerol + CoA

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

• hexadecanedioateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Production of Hexadecanedioylcarnitine
  3. hexadecanedioyl-CoA + H2O = hexadecanedioate + CoA + H(+)

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

• (2E,8Z,11Z,14Z)-eicosatetraenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)
  3. ELOVL fatty acid elongase (trans-2-cis,cis,cis-8,11,14-eicosatetraenoyl-CoA)

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

• 3-oxo-(11Z)-octadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (palmitolate)
  3. (9Z)-hexadecenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(11Z)-octadecenoyl-CoA + CO2 + CoA

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

• 3-oxo-(13Z)-eicosenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix
  3. (11Z)-octadecenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(13Z)-eicosenoyl-CoA + CO2 + CoA

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

• 20-hydroxy-(5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. L-cystathionine cysteine-lyase (deaminating
  2. cystine to cysteine conversion
  3. Resistance-Nodulation-Cell Division (Rnd) Tcdb:2.A.60.1.2

  Catalysed by CTH, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• 1-(9Z-octadecenoyl)-2-dodecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol)Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-(1'-sn-glycerol) + dodecanoyl-CoA = 1-(9Z-octadecenoyl)-2-dodecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol) + CoA

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

• 1-hexadecanoyl-2-dodecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol)Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. 1-hexadecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol) + dodecanoyl-CoA = 1-hexadecanoyl-2-dodecanoyl-sn-glycero-3-phospho-(1'-sn-glycerol) + CoA

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

• O-dodecanoyl-R-carnitineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Carnitine O-Palmitoyltransferase
  3. carnitine O-palmitoyltransferase (dodecanoylcarnitine)

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 11 routes

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

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

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

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

• (11Z,14Z)-eicosadienoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)
  3. (11Z,14Z)-eicosadienoyl-CoA hydrolase

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

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

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

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (DPA)
  3. (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoA hydrolase

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

• (7Z,10Z,13Z,16Z)-docosatetraenoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (adrenic acid)
  3. (7Z,10Z,13Z,16Z)-docosatetraenoyl-CoA hydrolase

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

• 2,6,10,14-tetramethylpentadecanoateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. fatty-acid-CoA ligase
  3. peroxisomal acyl-CoA thioesterase

  Catalysed by NAGS, SLC27A2, ACOT2mitochondrial matrix, cytosol, peroxisomeOne of 2 routes

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

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

• a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-O-(1Z-alkenyl)-sn-glycero-3-phosphocholine + an acyl-CoA = a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphocholine + CoA

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

• a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-O-(1Z-alkenyl)-sn-glycero-3-phosphoethanolamine + an acyl-CoA = a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphoethanolamine + CoA

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

• 1-O-(1Z)-alkenyl-2-octanoyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. a 1-O-(1Z-alkenyl)-sn-glycero-3-phosphoethanolamine + octanoyl-CoA = 1-O-(1Z)-alkenyl-2-octanoyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by NAGS, CROT, LPCAT4mitochondrial matrix

• 1-O-(1Z)-alkenyl-2-octadecanoyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. a 1-O-(1Z-alkenyl)-sn-glycero-3-phosphoethanolamine + octadecanoyl-CoA = 1-O-(1Z)-alkenyl-2-octadecanoyl-sn-glycero-3-phosphoethanolamine + CoA

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

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

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

• (2E,9Z)-hexadecadienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. long-chain-fatty-acid-CoA ligase (palmitolate)
  3. medium-chain acyl-CoA dehydrogenase (palmitoleoyl-CoA)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosolOne of 3 routes

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

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosolOne of 2 routes

• (2E,9Z)-octadecadienoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cystathionine)
  2. Transport of octadecenoyl-CoA into Mitochondrial Matrix
  3. medium-chain acyl-CoA dehydrogenase (oleoyl-CoA)

  Catalysed by NAGS, CPT1A, CPT1C, CPT1B and 4 moremitochondrial matrix, cytosolOne of 2 routes

• (2E,9Z,12Z)-octadecatrienoyl-CoAReach 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 + oxidized [electron-transfer flavoprotein] + H(+) = (2E,9Z,12Z)-octadecatrienoyl-CoA + reduced [electron-transfer flavoprotein]

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