an N-acylsphing-4-enine

Three steps

• (5Z)-dodecenoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

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

• O-[(5Z),(8Z),(11Z),(14Z)-eicosatetraenoyl]-beta-D-glucuronateReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + UDP-alpha-D-glucuronate = O-[(5Z),(8Z),(11Z),(14Z)-eicosatetraenoyl]-beta-D-glucuronate + UDP

  Catalysed by ASAH1, UGT1A9, UGT2B7, UGT1A6 and 3 morelysosome, extracellular space

• 3-oxo-(9Z)-eicosenoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. very-long-chain 3-oxoacyl-CoA synthase ((7Z)-octadecenoyl-CoA)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 moreGolgi apparatus, cytosol

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 moreGolgi apparatus, mitochondrial matrixOne of 5 routes

• (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. (6Z,9Z,12Z)-octadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 moreGolgi apparatus, cytosolOne of 2 routes

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

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

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

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

• (5Z)-octenoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

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

• a long-chain alcohol wax 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. a long chain fatty alcohol + a fatty acyl-CoA = a long-chain alcohol wax ester + CoA

  Catalysed by NAAA, ASAH1, ACER2, ACER1 and 5 more

• octanoateReach 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. beta-D-glucosyl-N-octanoylsphing-4E-enine + cholesterol = N-octanoylsphing-4-enine + cholesteryl 3-beta-D-glucoside
  3. N-octanoylsphing-4-enine + H2O = octanoate + sphing-4-enine

  Catalysed by GBA1, GBA2, ASAH1, ASAH2

• N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-ethanolamineReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. fatty acylamide amidohydrolase

  Catalysed by ASAH1, FAAHlysosome, extracellular space, cytosol

• CHEBI:27540Reach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 moreGolgi apparatus, mitochondrial matrix, cytosolOne of 6 routes

• beta-D-galactoseReach from here →
  1. UDP-alpha-D-galactose:N-acylsphingosine D-galactosyltransferase
  2. D-galactosyl-N-acylsphingosine galactohydrolase
  3. galactose[n] = beta-D-galactose[n]

  Catalysed by UGT8, GALC, GALMcytosol, lysosome, nucleusOne of 2 routes

• a ganglioside GA2 (d18:1(4E))Reach from here →
  1. UDP-glucose:N-acylsphingosine D-glucosyltransferase
  2. glucosylceramide pool to LacCer pool conversion
  3. beta-1,4-N-acetyl-galactosaminyltransferase (LacCer pool)

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

• dodecanalReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. 2 H+[r] + lauric acid[r] + NADH[r] = H2O[r] + NAD+[r] + dodecanal[r]

  Catalysed by ASAH1, ALDH3A2lysosome, extracellular space, endoplasmic reticulumOne of 3 routes

• hexacosan-1-olReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. hexacosanoyl-CoA + 2 NADPH + 2 H(+) = hexacosan-1-ol + 2 NADP(+) + CoA

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

• 1-(9Z-octadecenoyl)-sn-glycero-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. 1,2-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 ASAH1, ACER2, ACER1, ASAH2 and 2 moreGolgi apparatusOne of 4 routes

• CHEBI:28701Reach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 moreGolgi apparatus, mitochondrial matrix, peroxisomeOne of 7 routes

• 17alpha-hydroxypregnenoloneReach 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. cytochrome P450 (pregnenolone)

  Catalysed by GBA1, GBA2, CYP11A1, CYP17A1cytosolOne of 3 routes

• acetateReach 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. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate

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

• (9Z,12Z)-octadecadienoateReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. (9Z,12Z)-octadecadienoyl-CoA + H2O = (9Z,12Z)-octadecadienoate + CoA + H(+)

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

• a 1-O-alkyl-sn-glycero-3-phosphocholineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. Acyl-CoA:1-alkyl-sn-glycero-3-phosphocholine O-acyltransferase
  3. a 1-O-alkyl-2-acyl-sn-glycero-3-phosphocholine + H2O = a 1-O-alkyl-sn-glycero-3-phosphocholine + a fatty acid + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 moreGolgi apparatus, cytosolOne of 4 routes

• docosan-1-olReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. docosanoyl-CoA + 2 NADPH + 2 H(+) = docosan-1-ol + 2 NADP(+) + CoA

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

• octadecan-1-olReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. octadecanoyl-CoA + 2 NADPH + 2 H(+) = octadecan-1-ol + 2 NADP(+) + CoA

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

• 1-O-hexadecyl-2-(9Z)-octadecenoyl-sn-glycero-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 + (9Z)-octadecenoyl-CoA = 1-O-hexadecyl-2-(9Z)-octadecenoyl-sn-glycero-3-phosphocholine + CoA

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

• 1-O-hexadecyl-sn-glycerolReach 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 + H2O = 1-O-hexadecyl-sn-glycerol + phosphocholine + H(+)

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

• a 1-acylglycerolReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. a 2-acylglycerol + an acyl-CoA = a 1,2-diacylglycerol + CoA
  3. a 1,2-diacylglycerol + H2O = a 1-acylglycerol + a fatty acid + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 6 moreGolgi apparatusOne of 2 routes

• a sterol esterReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. a 2-acyl-sn-glycero-3-phosphocholine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phosphocholine + CoA
  3. a sterol + a 1,2-diacyl-sn-glycero-3-phosphocholine = a sterol ester + a 1-acyl-sn-glycero-3-phosphocholine

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

• 12-hydroxydodecanoateReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. alkane 1-monooxygenase (lauric acid)

  Catalysed by ASAH1, CYP4A11, CYP4A22lysosome, extracellular space, cytosolOne of 2 routes

• alpha-lactoseReach from here →
  1. UDP-glucose:N-acylsphingosine D-glucosyltransferase
  2. D-Glucosyl-N-acylsphingosine glucohydrolase
  3. UDP-alpha-D-galactose:D-glucose 4-beta-D-galactosyltransferase

  Catalysed by UGCG, GBA1, GBA3, GBA2 and 3 morecytosol, Golgi apparatus

• a 1-O-alkyl-2-acetyl-sn-glycero-3-phosphocholineReach from here →
  1. ceramide:phosphatidylcholine cholinephosphotransferase
  2. ceramide cholinephosphotransferase (CDP-choline)
  3. CDP-choline:1-alkyl-2-acetylglycerol cholinephosphotransferase

  Catalysed by SGMS1, SGMS2, CHPT1, CEPT1Golgi apparatus, endoplasmic reticulum, cytosol

• 7alpha,25-dihydroxycholesterolReach 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. cytochrome P450 (25-hydroxycholesterol)

  Catalysed by GBA1, GBA2, CH25H, CYP7B1endoplasmic reticulumOne of 2 routes

• (24S)-7alpha-dihydroxycholesterolReach 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 + reduced [NADPH--hemoprotein reductase] + O2 = 7alpha-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)
  3. 7alpha-hydroxycholesterol + reduced [NADPH--hemoprotein reductase] + O2 = (24S)-7alpha-dihydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by GBA1, GBA2, CYP7A1, CYP46A1One of 4 routes

• D-glucopyranose 6-phosphateReach from here →
  1. UDP-glucose:N-acylsphingosine D-glucosyltransferase
  2. D-Glucosyl-N-acylsphingosine glucohydrolase
  3. ATP:D-glucose 6-phosphotransferase

  Catalysed by UGCG, GBA1, GBA3, GBA2 and 5 morecytosolOne of 5 routes

• 1-O-hexadecyl-2-acetyl-sn-glycero-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 + acetyl-CoA = 1-O-hexadecyl-2-acetyl-sn-glycero-3-phosphocholine + CoA

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

• N-acetyl-beta-neuraminic acidReach from here →
  1. an N-acylsphing-4-enine + UDP-alpha-D-galactose = a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + UDP + H(+)
  2. a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + CMP-N-acetyl-beta-neuraminate = a ganglioside GM4 (d18:1(4E)) + CMP + H(+)
  3. Acylneuraminyl hydrolase

  Catalysed by UGT8, ST3GAL5, NEU4, NEU1 and 2 morelysosome

• taurineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

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

• 2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-glycerolReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. fatty acid amide hydrolase (2-arachidonoylglycerol)

  Catalysed by ASAH1, FAAHlysosome, extracellular space, cytosol

• an isogloboside iGb3Cer (d18:1(4E))Reach from here →
  1. UDP-glucose:N-acylsphingosine D-glucosyltransferase
  2. glucosylceramide pool to LacCer pool conversion
  3. a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) + UDP-alpha-D-galactose = an isogloboside iGb3Cer (d18:1(4E)) + UDP + H(+)

  Catalysed by UGCG, ALG1L2, B4GALT5, B3GALT2 and 33 morecytosol, endoplasmic reticulum

• N-tetracosanoylsphinganineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. tetracosanoyl-CoA + sphinganine = N-tetracosanoylsphinganine + CoA + H(+)

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

• N-hexacosanoylsphinganineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. hexacosanoyl-CoA + sphinganine = N-hexacosanoylsphinganine + CoA + H(+)

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

• 1,2,3-tri-(9Z-octadecenoyl)-glycerolReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. 1,2-di-(9Z-octadecenoyl)-glycerol + (9Z)-octadecenoate + H(+) = 1,2,3-tri-(9Z-octadecenoyl)-glycerol + H2O

  Catalysed by ASAH1, DGAT1, LIPA, PNPLA4 and 2 morelysosome, extracellular space

• 16-hydroxyhexadecanoateReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. Fatty Acid Omega Oxidation (C16->W-Ohc16), Endoplasmatic Reticulum

  Catalysed by ASAH1, CYP4F2, CYP4F3lysosome, extracellular space, endoplasmic reticulumOne of 2 routes

• 1-O-hexadecyl-2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-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 + (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA = 1-O-hexadecyl-2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphocholine + CoA

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

• a 6-(N-acetyl-alpha-D-glucosaminyl)-1-(1,2-diacyl-sn-glycero-3-phospho)-1D-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 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol) + UDP-N-acetyl-alpha-D-glucosamine = a 6-(N-acetyl-alpha-D-glucosaminyl)-1-(1,2-diacyl-sn-glycero-3-phospho)-1D-myo-inositol + UDP + H(+)

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

• glycineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. glycine N-acyltransferase (N-arachidonoylglycine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 moreGolgi apparatus, mitochondrial matrixOne of 10 routes

• a (3S)-3-hydroxyacyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. an acyl-CoA + acetyl-CoA = a 3-oxoacyl-CoA + CoA
  3. a (3S)-3-hydroxyacyl-CoA + NAD(+) = a 3-oxoacyl-CoA + NADH + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 moreGolgi apparatus

• (R)-methylmalonyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

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

• (S)-methylmalonyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. (S)-methylmalonyl-CoA hydrolase

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

• 3'-dephospho-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. acid phosphatase 2, lysosomal (CoA)

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