an N-acylsphinganine

Three steps

• 3-hydroxyisovaleryl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 9 morecytosol

• CHEBI:63544Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 10 routes

• 18-hydroxy-(5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. cytochrome P450 (arachidonate)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 morecytosol, extracellular space, endoplasmic reticulumOne of 2 routes

• a 1-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. an N-acylsphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acylsphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. CDP-ethanolamine + a 1,2-diacyl-sn-glycerol = a 1,2-diacyl-sn-glycero-3-phosphoethanolamine + CMP + H(+)
  3. N-(acetyl)-sphing-4-enine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = 1-O-acyl-N-(acetyl)-sphing-4-enine + a 1-acyl-sn-glycero-3-phosphoethanolamine

  Catalysed by SGMS2, SAMD8, SELENOI, CEPT1 and 1 moreOne of 2 routes

• 1-O-hexadecyl-sn-glycero-3-phosphocholineReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-O-hexadecyl-2-acetyl-sn-glycero-3-phosphocholine + sphing-4-enine = 1-O-hexadecyl-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine + H(+)

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 3 morecytosol, Golgi apparatus

• a 1,2-diacyl-sn-glycero-3-phospho-N-methylethanolamineReach from here →
  1. an N-acylsphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acylsphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. CDP-ethanolamine + a 1,2-diacyl-sn-glycerol = a 1,2-diacyl-sn-glycero-3-phosphoethanolamine + CMP + H(+)
  3. a 1,2-diacyl-sn-glycero-3-phosphoethanolamine + S-adenosyl-L-methionine = a 1,2-diacyl-sn-glycero-3-phospho-N-methylethanolamine + S-adenosyl-L-homocysteine + H(+)

  Catalysed by SGMS2, SAMD8, SELENOI, CEPT1 and 1 more

• a 1-acyl-sn-glycerolReach from here →
  1. an N-acylsphinganine + H2O = sphinganine + a fatty acid
  2. a 1,3-diacyl-sn-glycerol + H2O = a 1-acyl-sn-glycerol + a fatty acid + H(+)
  3. a 1,3-diacyl-sn-glycerol + H2O = a 1-acyl-sn-glycerol + a fatty acid + H(+)

  Catalysed by ACER2, ACER1, ACER3, ABHD11

• a 1,2-diacyl-sn-glycero-3-phospho-(1'-sn-glycerol)Reach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-acyl-sn-glycero-3-phospho-(1'-sn-glycerol) + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-(1'-sn-glycerol) + CoA

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 5 morecytosol, Golgi apparatusOne of 2 routes

• (4R)-hydroxysphinganine 1-phosphateReach from here →
  1. dihydroceramide,ferrocytochrome b5:oxygen oxidoreductase (C4-hydroxylating)
  2. Phytoceramide amidohydrolase
  3. Sphingosine Kinase 1

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 4 morecytosolOne of 2 routes

• hexacosanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (cerotic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 9 routes

• a 2-acyl-sn-glycerol 3-phosphateReach from here →
  1. an N-acylsphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acylsphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. a 1,2-diacyl-sn-glycerol + ATP = a 1,2-diacyl-sn-glycero-3-phosphate + ADP + H(+)
  3. a 1,2-diacyl-sn-glycero-3-phosphate + H2O = a 2-acyl-sn-glycerol 3-phosphate + a fatty acid + H(+)

  Catalysed by SGMS2, SAMD8, DGKI, DGKA and 10 moreOne of 2 routes

• tetracosanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (lignocerate)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 17 routes

• docosanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (behenic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 14 routes

• a 2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. an N-acylsphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acylsphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. CDP-ethanolamine + a 1,2-diacyl-sn-glycerol = a 1,2-diacyl-sn-glycero-3-phosphoethanolamine + CMP + H(+)
  3. a 1,2-diacyl-sn-glycero-3-phosphoethanolamine + H2O = a 2-acyl-sn-glycero-3-phosphoethanolamine + a fatty acid + H(+)

  Catalysed by SGMS2, SAMD8, SELENOI, CEPT1 and 1 moreOne of 2 routes

• (22R)-hydroxycholesterolReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. 2 reduced [adrenodoxin] + cholesterol + O2 + 2 H(+) = (22R)-hydroxycholesterol + 2 oxidized [adrenodoxin] + H2O

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 1 morecytosolOne of 2 routes

• (2E)-octadecenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 morecytosolOne of 4 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 9 routes

• 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-(1'-sn-glycerol)Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. 1-(9Z-octadecenoyl)-sn-glycero-3-phospho-(1'-sn-glycerol) + H2O = sn-glycero-3-phospho-(1'-sn-glycerol) + (9Z)-octadecenoate + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 morecytosol, extracellular space

• 1-hexadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phospho-(1D-myo-inositol)Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. 1-hexadecanoyl-2-(9Z-octadecenoyl)-sn-glycero-3-phospho-(1D-myo-inositol) + H2O = 1-hexadecanoyl-sn-glycero-3-phospho-(1D-myo-inositol) + (9Z)-octadecenoate + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 morecytosol, extracellular space

• 1,2-dihexadecanoyl-sn-glycero-3-phosphateReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. 1,2-dihexadecanoyl-sn-glycero-3-phosphocholine + an N-acylsphing-4-enine = 1,2-dihexadecanoyl-sn-glycerol + a sphingomyelin
  3. 1,2-dihexadecanoyl-sn-glycerol + ATP = 1,2-dihexadecanoyl-sn-glycero-3-phosphate + ADP + H(+)

  Catalysed by DEGS1, DEGS2, SGMS2, DGKAcytosol

• N-dodecanoylsphing-4-enineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. N-dodecanoylsphing-4-enine + H2O = dodecanoate + sphing-4-enine

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 morecytosol, extracellular spaceOne of 4 routes

• N-tetradecanoylsphing-4-enineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. N-tetradecanoylsphing-4-enine + H2O = sphing-4-enine + tetradecanoate

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 morecytosol, extracellular spaceOne of 2 routes

• N-hexadecanoylsphing-4-enineReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + hexadecanoyl-CoA = N-hexadecanoylsphing-4-enine + CoA + H(+)

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 6 morecytosol, Golgi apparatusOne of 3 routes

• N-octadecanoylsphing-4-enineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. N-octadecanoylsphing-4-enine + H2O = sphing-4-enine + octadecanoate

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 morecytosol, extracellular spaceOne of 4 routes

• N-eicosanoyl-sphing-4-enineReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. eicosanoyl-CoA + sphing-4-enine = N-eicosanoyl-sphing-4-enine + CoA + H(+)

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 4 morecytosol, Golgi apparatus

• N-tetracosanoyl-sphing-4-enineReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. tetracosanoyl-CoA + sphing-4-enine = N-tetracosanoyl-sphing-4-enine + CoA + H(+)

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 3 morecytosol, Golgi apparatus

• 1-hexadecanoyl-sn-glycero-3-phosphocholineReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. 1-hexadecanoyl-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphocholine + cholesterol = cholesteryl (5Z,8Z,11Z,14Z)-eicosatetraenoate + 1-hexadecanoyl-sn-glycero-3-phosphocholine

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 1 morecytosolOne of 9 routes

• 1,2-dihexadecanoyl-sn-glycero-3-phosphocholineReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. 1,2-dihexadecanoyl-sn-glycero-3-phosphocholine + an N-acylsphing-4-enine = 1,2-dihexadecanoyl-sn-glycerol + a sphingomyelin

  Catalysed by DEGS1, DEGS2, SGMS1, SGMS2cytosol, Golgi apparatusOne of 2 routes

• (7Z,10Z,13Z,16Z)-docosatetraenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (7Z,10Z,13Z,16Z)-docosatetraenoate + ATP + CoA = (7Z,10Z,13Z,16Z)-docosatetraenoyl-CoA + AMP + diphosphate

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 morecytosol, extracellular space

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 13 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 9 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (linolenate)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 10 routes

• (13Z)-docosenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 7 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 10 routes

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 6 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 5 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 9 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 7 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 8 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 6 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (DHA)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 11 routes

• heptadecanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 7 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 6 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 6 routes

• N-(15Z-tetracosenoyl)-sphing-4-enineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. N-(15Z-tetracosenoyl)-sphing-4-enine + H2O = (15Z)-tetracosenoate + sphing-4-enine

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 morecytosol, extracellular spaceOne of 3 routes

• N-(tetracosenoyl)-sphing-4-enineReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. tetracosenoyl-CoA + sphing-4-enine = N-(tetracosenoyl)-sphing-4-enine + CoA + H(+)

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 3 morecytosol, Golgi apparatus

• nonadecanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 7 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 morecytosolOne of 3 routes

• (12R)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + O2 = (12R)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 morecytosol, extracellular space

• (8S)-hydroperoxy-(5Z,9E,11Z,14Z)-eicosatetraenoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. Arachidonate:oxygen 8-oxidoreductase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 morecytosol, extracellular spaceOne of 2 routes