an N-acylsphinganine

Three steps

• isobutyryl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 morecytosol, mitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 8 morecytosol, mitochondrial matrix

• isovaleryl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 morecytosol, mitochondrial matrixOne of 3 routes

• arachidonoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Arachidonate:CoA ligase (AMP-forming)

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

• CHEBI:15519Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. propanoyl-CoA C-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 morecytosol, peroxisomeOne of 2 routes

• palmitoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Palmitate:CoA ligase (AMP-forming)

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

• oleoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Transport of octadecenoyl-CoA into Mitochondrial Matrix

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

• phenylacetyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Phenylacetate:CoA ligase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 morecytosolOne of 3 routes

• propionyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Propanoate:CoA ligase (AMP-forming)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 morecytosolOne of 43 routes

• (7S)-hydroperoxy-(4Z,8E,10Z,13Z,16Z,19Z)-docosahexaenoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate + O2 = (7S)-hydroperoxy-(4Z,8E,10Z,13Z,16Z,19Z)-docosahexaenoate

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

• (11S)-hydroperoxy-(4Z,7Z,9E,13Z,16Z,19Z)-docosahexaenoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate + O2 = (11S)-hydroperoxy-(4Z,7Z,9E,13Z,16Z,19Z)-docosahexaenoate

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

• 12(S)-HPETEReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. arachidonate:oxygen 12-oxidoreductase

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

• 15(S)-HPETEReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. arachidonate:oxygen 15-oxidoreductase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 morecytosol, extracellular spaceOne of 4 routes

• 5(S)-HPETEReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. Arachidonate:oxygen 5-oxidoreductase

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

• a 1-hexadecanoyl-2-acylglycerolReach 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 + H2O = a 2-acylglycerol + a fatty acid + H(+)
  3. a 2-acylglycerol + hexadecanoyl-CoA = a 1-hexadecanoyl-2-acylglycerol + CoA

  Catalysed by SGMS2, SAMD8, DAGLB, ABHD11 and 2 more

• CHEBI:15650Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 39 morecytosolOne of 2 routes

• CHEBI:15727Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 8 morecytosol

• CHEBI:15746Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 9 morecytosolOne of 2 routes

• 1-acylglycerone 3-phosphateReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:glycerone-phosphate O-acyltransferase

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

• 11-cis-retinyl hexadecanoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. 11-peroxy-docosahexaenoate to DHA conversion

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

• 11-cis-retinolReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 12 morecytosolOne of 3 routes

• CHEBI:16436Reach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. ceramide cholinephosphotransferase (CDP-choline)

  Catalysed by DEGS1, DEGS2, SGMS1, SGMS2cytosol, Golgi apparatus, endoplasmic reticulum

• oxaloacetateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. ATP citrate lyase (citrate)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 8 morecytosol

• pregnenoloneReach 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. 6 reduced [adrenodoxin] + cholesterol + 3 O2 + 6 H(+) = 4-methylpentanal + pregnenolone + 6 oxidized [adrenodoxin] + 4 H2O

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 1 morecytosol

• 8-hydroxydodecanoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. dodecanoate + reduced [NADPH--hemoprotein reductase] + O2 = 8-hydroxydodecanoate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

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

• 10-hydroxydodecanoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. dodecanoate + reduced [NADPH--hemoprotein reductase] + O2 = 10-hydroxydodecanoate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

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

• 9-hydroxydodecanoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. dodecanoate + reduced [NADPH--hemoprotein reductase] + O2 = 9-hydroxydodecanoate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

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

• 9-hydroxytetradecanoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. tetradecanoate + reduced [NADPH--hemoprotein reductase] + O2 = 9-hydroxytetradecanoate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

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

• 10-hydroxytetradecanoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. tetradecanoate + reduced [NADPH--hemoprotein reductase] + O2 = 10-hydroxytetradecanoate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

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

• 12-hydroxytetradecanoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. tetradecanoate + reduced [NADPH--hemoprotein reductase] + O2 = 12-hydroxytetradecanoate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

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

• 11-hydroxytetradecanoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. tetradecanoate + reduced [NADPH--hemoprotein reductase] + O2 = 11-hydroxytetradecanoate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

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

• CHEBI:16856Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. transport of arachidonate and GSH (cytosol to extracellular)

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

• psychosineReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 2 morecytosol, Golgi apparatus

• 1-acyl-sn-glycerol 3-phosphateReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 6 morecytosol, Golgi apparatus, mitochondrial matrixOne of 7 routes

• a cholesterol esterReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + cholesterol = a cholesterol ester + CoA

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 4 morecytosol, Golgi apparatusOne of 4 routes

• octadecanalReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. octadecanal + NAD(+) + H2O = octadecanoate + NADH + 2 H(+)

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

• anilineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Acetyl-CoA:arylamine N-acetyltransferase

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

• CHEBI:17312Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 morecytosolOne of 2 routes

• all-trans-retinolReach from here →
  1. an N-acylsphinganine + H2O = sphinganine + a fatty acid
  2. an all-trans-retinyl ester + H2O = 11-cis-retinol + a fatty acid + H(+)
  3. carboxylesterase (retinyl-ester)

  Catalysed by ACER2, ACER1, ACER3, RPE65 and 20 morecytosolOne of 2 routes

• 1-(alk-1-enyl)-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-alkenylglycerophosphoethanolamine O-acyltransferase

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 6 morecytosol, Golgi apparatus

• cholesteryl 3-beta-D-glucosideReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. an N-acylsphing-4-enine + UDP-alpha-D-glucose = a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + UDP + H(+)
  3. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine

  Catalysed by DEGS1, DEGS2, UGCG, GBA1 and 1 morecytosolOne of 8 routes

• 7alpha-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. cholesterol + reduced [NADPH--hemoprotein reductase] + O2 = 7alpha-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 1 morecytosol

• 1-O-acyl-sn-glycero-3-phosphocholine(1+)Reach 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. diacylglycerol cholinephosphotransferase (1,2-diacylglycerol-LD-PC pool)
  3. phosphatidylcholine 2-acylhydrolase

  Catalysed by SGMS2, SAMD8, CHPT1, CEPT1 and 18 morecytosol, endoplasmic reticulumOne of 8 routes

• O-phosphoethanolamineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. ATP:sphinganine 1-phosphotransferase
  3. Sphinganine-1-phosphate pamlmitaldehyde-lyase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 morecytosol, endoplasmic reticulum

• L-cysteineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 8 morecytosol, mitochondrial matrix

• hexadecanalReach from here →
  1. N-acylsphingosine amidohydrolase
  2. ATP:sphinganine 1-phosphotransferase
  3. Sphinganine-1-phosphate pamlmitaldehyde-lyase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 morecytosol, endoplasmic reticulumOne of 4 routes

• 26-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. cholesterol + reduced [NADPH--hemoprotein reductase] + O2 = 26-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 1 morecytosol

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 morecytosol, mitochondrial matrix

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

  Catalysed by ACER2, ACER1, ACER3, LIPEOne of 2 routes

• CHEBI:17927Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 morecytosol