pyruvate

Three steps

• ganglioside GM1 (d18:1(4E)/18:0)Reach from here →
  1. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. ganglioside GA1 (d18:1(4E)/18:0) + CMP-N-acetyl-beta-neuraminate = ganglioside GM1 (d18:1(4E)/18:0) + CMP + H(+)

  Catalysed by NPL, CMAS, ST3GAL5cytosol, nucleus

• L-threonylcarbamoyladenylateReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)
  3. L-threonine + hydrogencarbonate + ATP = L-threonylcarbamoyladenylate + diphosphate + H2O

  Catalysed by AGXT2, SLC7A10, YRDCmitochondrial matrix, inner side of the membrane, outer side of the membrane

• 1-octadecanoyl-sn-glycero-3-phosphocholineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-octadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 1-(9Z-octadecenoyl)-N-(acetyl)-sphing-4-enine + 1-octadecanoyl-sn-glycero-3-phosphocholine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosolOne of 3 routes

• 4-acetamidobutanalReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-acetylputrescine + H2O = putrescine + acetate
  3. N-Acetylputrescine:oxygen oxidoreductase(deaminating)(flavin-containing)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 7 moreextracellular space, cytosolOne of 2 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (DHA)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• heptadecanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• nonadecanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes

• 1-(9Z-octadecenoyl)-N-(acetyl)-sphing-4-enineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1,2-di-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 1-(9Z-octadecenoyl)-N-(acetyl)-sphing-4-enine + 1-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosolOne of 8 routes

• 2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-hexadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 1-hexadecanoyl-N-(acetyl)-sphing-4-enine + 2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosolOne of 2 routes

• 1-octadecanoyl-N-(acetyl)-sphing-4-enineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-octadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 1-octadecanoyl-N-(acetyl)-sphing-4-enine + 2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosolOne of 5 routes

• 2-octadecanoyl-sn-glycero-3-phosphocholineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-(9Z)-octadecenoyl-2-octadecanoyl-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 2-octadecanoyl-sn-glycero-3-phosphocholine + 1-(9Z-octadecenoyl)-N-(acetyl)-sphing-4-enine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosol

• 1-hexadecanoyl-N-(acetyl)-sphing-4-enineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-hexadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 1-hexadecanoyl-N-(acetyl)-sphing-4-enine + 2-(9Z-octadecenoyl)-sn-glycero-3-phosphocholine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosolOne of 9 routes

• 2-hexadecanoyl-sn-glycero-3-phosphocholineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-(9Z-octadecenoyl)-2-hexadecanoyl-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 1-(9Z-octadecenoyl)-N-(acetyl)-sphing-4-enine + 2-hexadecanoyl-sn-glycero-3-phosphocholine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosol

• 2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphocholineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-hexadecanoyl-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 1-hexadecanoyl-N-(acetyl)-sphing-4-enine + 2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphocholine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosolOne of 2 routes

• 1-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-N-(acetyl)-sphing-4-enineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-hexadecanoyl-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 1-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-N-(acetyl)-sphing-4-enine + 1-hexadecanoyl-sn-glycero-3-phosphocholine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosolOne of 3 routes

• 2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphocholineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-hexadecanoyl-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphocholine + 1-hexadecanoyl-N-(acetyl)-sphing-4-enine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosol

• 2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-hexadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholine + 1-hexadecanoyl-N-(acetyl)-sphing-4-enine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosol

• 1-(9Z,12Z-octadecadienoyl)-N-acetylsphing-4-enineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-hexadecanoyl-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 1-(9Z,12Z-octadecadienoyl)-N-acetylsphing-4-enine + 1-hexadecanoyl-sn-glycero-3-phosphocholine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosolOne of 3 routes

• 1-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-N-(acetyl)-sphing-4-enineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-hexadecanoyl-2-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine = 1-(4Z,7Z,10Z,13Z,16Z,19Z-docosahexaenoyl)-N-(acetyl)-sphing-4-enine + 1-hexadecanoyl-sn-glycero-3-phosphocholine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosol

• 2-(9Z-octadecenoyl)-sn-glycero-3-phosphoethanolamineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-hexadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phosphoethanolamine + N-(acetyl)-sphing-4-enine = 2-(9Z-octadecenoyl)-sn-glycero-3-phosphoethanolamine + 1-hexadecanoyl-N-(acetyl)-sphing-4-enine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosol

• 2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-hexadecanoyl-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphoethanolamine + N-(acetyl)-sphing-4-enine = 2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphoethanolamine + 1-hexadecanoyl-N-(acetyl)-sphing-4-enine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosol

• 2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. 1-hexadecanoyl-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphoethanolamine + N-(acetyl)-sphing-4-enine = 2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphoethanolamine + 1-hexadecanoyl-N-(acetyl)-sphing-4-enine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosol

• 16alpha,17alpha-dihydroxyprogesteroneReach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. cytochrome P450 (17alpha-hydroxyprogesterone)
  3. 17alpha-hydroxyprogesterone + reduced [NADPH--hemoprotein reductase] + O2 = 16alpha,17alpha-dihydroxyprogesterone + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 2 moreextracellular space, cytosol, endoplasmic reticulum

• dodecanedioyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Activation of dodecanedioic acid

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Production of Sebacoylcarnitine

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Production of Suberylcarnitine

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by AGXT2mitochondrial matrix, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• 2-acyl-sn-glycero-3-phospho-(1'-sn-glycerol)Reach from here →
  1. transport of acetate and pyruvate (cytosol to extracellular)
  2. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate
  3. a 1,2-diacyl-sn-glycero-3-phospho-(1'-sn-glycerol) + N-(acetyl)-sphing-4-enine = 2-acyl-sn-glycero-3-phospho-(1'-sn-glycerol) + 1-O-acyl-N-(acetyl)-sphing-4-enine

  Catalysed by SLC16A4, SLC16A5, SLC16A3, SLC16A7 and 3 moreextracellular space, cytosol

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by AGXT2, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes

• beta-citrylglutamateReach from here →
  1. Pyruvate:carbon-dioxide ligase (ADP-forming)
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. citrate + L-glutamate + ATP = beta-citrylglutamate + ADP + phosphate + H(+)

  Catalysed by PC, CS, RIMKLBmitochondrial matrix

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by AGXT2, CRAT, CROTmitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by AGXT2, ACAA1, HSD17B4, EHHADH and 1 moremitochondrial matrix, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Production of Hexadecanedioylcarnitine

  Catalysed by AGXT2, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 2 routes