an N-acylsphing-4-enine

Three steps

• 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, CYP4Z1lysosome, 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, CYP4Z1lysosome, 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, CYP4Z1lysosome, 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, CYP4Z1lysosome, 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, CYP4Z1lysosome, 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, CYP4Z1lysosome, 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, CYP4Z1lysosome, extracellular space

• galactitolReach from here →
  1. UDP-alpha-D-galactose:N-acylsphingosine D-galactosyltransferase
  2. D-galactosyl-N-acylsphingosine galactohydrolase
  3. galactitol:NADP+ 1-oxidoreductase

  Catalysed by UGT8, GALC, AKR1B1cytosol, lysosomeOne of 2 routes

• formaldehydeReach from here →
  1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  2. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O
  3. N(6),N(6)-dimethyl-L-lysyl(4)-[histone H3] + 2 A + 2 H2O = L-lysyl(4)-[histone H3] + 2 formaldehyde + 2 AH2

  Catalysed by GBA1, GBA2, CH25H, KDM1A and 1 moreOne of 3 routes

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

  Catalysed by ASAH1lysosome, extracellular space, cytosolOne of 2 routes

• sn-glycerol 3-phosphocholineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. 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(+)
  3. 1-O-hexadecyl-sn-glycero-3-phosphocholine + 1-hexadecanoyl-sn-glycero-3-phosphocholine = 1-O-hexadecyl-2-hexadecanoyl-sn-glycero-3-phosphocholine + sn-glycerol 3-phosphocholine

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 moreGolgi apparatusOne of 8 routes

• progesteroneReach from here →
  1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  2. 6 reduced [adrenodoxin] + cholesterol + 3 O2 + 6 H(+) = 4-methylpentanal + pregnenolone + 6 oxidized [adrenodoxin] + 4 H2O
  3. Pregnenolone:NAD+ 3-oxidoreductase

  Catalysed by GBA1, GBA2, CYP11A1, HSD3B1 and 1 morecytosolOne of 2 routes

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

  Catalysed by ASAH1, ALDH3A2lysosome, extracellular space

• a beta-D-GlcNAc-(1->3)-beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))Reach from here →
  1. UDP-glucose:N-acylsphingosine D-glucosyltransferase
  2. glucosylceramide pool to LacCer pool conversion
  3. UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase (LacCer pool)

  Catalysed by UGCG, ALG1L2, B4GALT5, B3GALT2 and 32 morecytosol, endoplasmic reticulumOne of 3 routes

• S-(9Z,12Z,15Z-octadecatrienoyl)-4'-phosphopantetheineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. (9Z,12Z,15Z)-octadecatrienoyl-CoA + H2O = S-(9Z,12Z,15Z-octadecatrienoyl)-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moreGolgi apparatus, cytosol

• S-(9Z,12Z-octadecadienoyl)-4'-phosphopantetheineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. (9Z,12Z)-octadecadienoyl-CoA + H2O = S-(9Z,12Z-octadecadienoyl)-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moreGolgi apparatus, cytosol

• S-(9Z-hexadecenoyl)-4'-phosphopantetheineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. (9Z)-hexadecenoyl-CoA + H2O = S-(9Z-hexadecenoyl)-4'-phosphopantetheine + adenosine 3',5'-bisphosphate + 2 H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moreGolgi apparatus, cytosol

• N-octadecanoyl-heptadecasphing-4-enineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. heptadecasphing-4-enine + octadecanoyl-CoA = N-octadecanoyl-heptadecasphing-4-enine + CoA + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moreGolgi apparatus, cytosol

• myo-inositolReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. a 1-acyl-sn-glycero-3-phospho-(1D-myo-inositol) + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol) + CoA
  3. a CDP-1,2-diacyl-sn-glycerol + myo-inositol = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol) + CMP + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 moreGolgi apparatus

• anilineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 6 moreGolgi apparatus, mitochondrial matrix, cytosol

• CHEBI:17312Reach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moreGolgi apparatus, mitochondrial matrix, cytosolOne of 2 routes

• all-trans-retinolReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. all-trans-retinol + an acyl-CoA = an all-trans-retinyl ester + CoA
  3. carboxylesterase (retinyl-ester)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 23 moreGolgi apparatus, cytosolOne of 3 routes

• O-hexadecanoyl-(R)-carnitineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. palmitoyl-CoA:L-carnitine O-palmitoyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 moreGolgi apparatus, cytosol, peroxisomeOne of 4 routes

• AH2Reach from here →
  1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  2. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O
  3. A + NADH + H(+) = AH2 + NAD(+)

  Catalysed by GBA1, GBA2, CH25H, AIFM1 and 1 moreOne of 19 routes

• 1-O-acyl-sn-glycero-3-phosphocholine(1+)Reach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. Acyl-CoA:1-acyl-sn-glycero-3-phosphocholine O-acyltransferase
  3. phosphatidylcholine 2-acylhydrolase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 22 moreGolgi apparatus, cytosol, endoplasmic reticulumOne of 8 routes

• O-phosphoethanolamineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. ATP:sphingosine 1-phosphotransferase
  3. sphingosine-1-phosphate palmitaldehyde-lyase (phosphoethanolamine-forming)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moreGolgi apparatus, cytosolOne of 2 routes

• L-cysteineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 moreGolgi apparatus, mitochondrial matrixOne of 2 routes

• (2E)-hexadecenalReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. ATP:sphingosine 1-phosphotransferase
  3. sphingosine-1-phosphate palmitaldehyde-lyase (phosphoethanolamine-forming)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moreGolgi apparatus, cytosolOne of 2 routes

• hexadecanalReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. 2 H+[c] + NADH[c] + palmitate[c] = H2O[c] + hexadecanal[c] + NAD+[c]

  Catalysed by ASAH1, ALDH3B1, ALDH3B2, ALDH3A2lysosome, extracellular space, cytosolOne of 2 routes

• all-trans-retinyl hexadecanoateReach from here →
  1. ceramide:phosphatidylcholine cholinephosphotransferase
  2. 1,2-dihexadecanoyl-sn-glycero-3-phosphocholine + an N-acylsphing-4-enine = 1,2-dihexadecanoyl-sn-glycerol + a sphingomyelin
  3. all-trans-retinol + 1,2-dihexadecanoyl-sn-glycero-3-phosphocholine = all-trans-retinyl hexadecanoate + 2-hexadecanoyl-sn-glycero-3-phosphocholine

  Catalysed by SGMS1, SGMS2, LRATGolgi apparatusOne of 2 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 moreGolgi apparatus, mitochondrial matrixOne of 2 routes

• lactoseReach from here →
  1. UDP-glucose:N-acylsphingosine D-glucosyltransferase
  2. D-Glucosyl-N-acylsphingosine glucohydrolase
  3. D-glucose + UDP-alpha-D-galactose = lactose + UDP + H(+)

  Catalysed by UGCG, GBA1, GBA3, GBA2 and 2 morecytosol

• glycerolReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. a 2-acylglycerol + an acyl-CoA = a 1,2-diacylglycerol + CoA
  3. a 1-acylglycerol + a 1,2-diacylglycerol = a triacylglycerol + glycerol

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 8 moreGolgi apparatusOne of 4 routes

• CHEBI:17808Reach from here →
  1. UDP-glucose:N-acylsphingosine D-glucosyltransferase
  2. D-Glucosyl-N-acylsphingosine glucohydrolase
  3. ITP:D-glucose 6-phosphotransferase

  Catalysed by UGCG, GBA1, GBA3, GBA2 and 4 morecytosol

• 7alpha-hydroxycholest-4-en-3-oneReach from here →
  1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  2. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O
  3. Cholest-5-Ene-3Beta, 7Alpha-Diol:NAD+ 3-Oxidoreductase Bile Acid Biosynthesis

  Catalysed by GBA1, GBA2, CH25H, HSD3B7endoplasmic reticulumOne of 5 routes

• D-glucitolReach from here →
  1. UDP-glucose:N-acylsphingosine D-glucosyltransferase
  2. D-Glucosyl-N-acylsphingosine glucohydrolase
  3. Aldehyde Reductase

  Catalysed by UGCG, GBA1, GBA3, GBA2cytosolOne of 3 routes

• CHEBI:17927Reach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moreGolgi apparatus, mitochondrial matrix, cytosol

• a diacylglycerolReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. a triacylglycerol + H2O = a diacylglycerol + a fatty acid + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 24 moreGolgi apparatus, cytosolOne of 2 routes

• L-glutamineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)
  3. an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 moreGolgi apparatus

• phosphatidyl-L-serineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. Acyl-CoA:1-acyl-sn-glycero-3-phosphocholine O-acyltransferase
  3. phosphatidylserine synthase (PC-LD pool)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 moreGolgi apparatus, cytosolOne of 2 routes

• a globoside Gb3Cer (d18:1(4E))Reach from here →
  1. UDP-glucose:N-acylsphingosine D-glucosyltransferase
  2. glucosylceramide pool to LacCer pool conversion
  3. alpha 1,4-galactosyltransferase (P blood group) (LacCer pool)

  Catalysed by UGCG, ALG1L2, B4GALT5, B3GALT2 and 33 morecytosol, endoplasmic reticulumOne of 3 routes

• (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + O2 = (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoA + H2O2

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moreGolgi apparatus, cytosol

• beta-D-xylosyl-(1<->1')-N-(9Z-octadecenoyl)-sphing-4-enineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. N-(9Z-octadecenoyl)-sphing-4-enine + H2O = sphing-4-enine + (9Z)-octadecenoate
  3. N-(9Z-octadecenoyl)-sphing-4-enine + UDP-alpha-D-xylose = beta-D-xylosyl-(1<->1')-N-(9Z-octadecenoyl)-sphing-4-enine + UDP + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 moreGolgi apparatus

• 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. a 2-acyl-sn-glycero-3-phosphoethanolamine + octadecanoyl-CoA = 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moreGolgi apparatus, cytosol

• 11-cis-retinyl esterReach from here →
  1. an N-acylsphing-4-enine + H2O = sphing-4-enine + a fatty acid
  2. a fatty acid + ATP + CoA = a fatty acyl-CoA + AMP + diphosphate
  3. 11-cis-retinol + a fatty acyl-CoA = 11-cis-retinyl ester + CoA

  Catalysed by NAAA, ASAH1, ACER2, ACER1 and 4 more

• 13-cis-retinyl esterReach from here →
  1. an N-acylsphing-4-enine + H2O = sphing-4-enine + a fatty acid
  2. a fatty acid + ATP + CoA = a fatty acyl-CoA + AMP + diphosphate
  3. 13-cis-retinol + a fatty acyl-CoA = 13-cis-retinyl ester + CoA

  Catalysed by NAAA, ASAH1, ACER2, ACER1 and 4 more

• 9-cis-retinyl esterReach from here →
  1. an N-acylsphing-4-enine + H2O = sphing-4-enine + a fatty acid
  2. a fatty acid + ATP + CoA = a fatty acyl-CoA + AMP + diphosphate
  3. 9-cis-retinol + a fatty acyl-CoA = 9-cis-retinyl ester + CoA

  Catalysed by NAAA, ASAH1, ACER2, ACER1 and 4 more

• (5S)-hydroperoxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoateReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoate + O2 = (5S)-hydroperoxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoate

  Catalysed by ASAH1, ALOX5lysosome, extracellular space

• 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. eicosanoyl-CoA + 2-(9Z-octadecenoyl)-glycerol = 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 moreGolgi apparatus, cytosol

• 1D-myo-inositol 1,4,5-trisphosphateReach from here →
  1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  2. a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol-4,5-bisphosphate)(out) + cholesterol(in) = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol-4,5-bisphosphate)(in) + cholesterol(out)
  3. a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol-4,5-bisphosphate) + H2O = 1D-myo-inositol 1,4,5-trisphosphate + a 1,2-diacyl-sn-glycerol + H(+)

  Catalysed by GBA1, GBA2, OSBP2, OSBPL2 and 13 moreinner side of the membrane, outer side of the membrane