an N-acylsphing-4-enine

Three steps

• (2E)-dodecenoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moreGolgi apparatus, mitochondrial matrixOne of 8 routes

• 3-oxohexadecanoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. tetradecanoyl-CoA + malonyl-CoA + H(+) = 3-oxohexadecanoyl-CoA + CO2 + CoA

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

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 6 moreGolgi apparatus, mitochondrial matrix, peroxisomeOne of 2 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. (8Z,11Z,14Z)-eicosatrienoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moreGolgi apparatus, cytosolOne of 2 routes

• butanoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 moreGolgi apparatus, mitochondrial matrix, cytosolOne of 9 routes

• glutaryl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. Production of Glutaryl Carnitine

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

• hexadecanoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. hexadecanoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (9Z)-hexadecenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

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

• itaconyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. Itaconate:CoA ligase (GDP-forming)

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

• octanoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 moreGolgi apparatus, mitochondrial matrixOne of 30 routes

• (9Z)-octadecenoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. octadecanoyl-CoA + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (9Z)-octadecenoyl-CoA + 2 Fe(III)-[cytochrome b5] + 2 H2O

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

• phytanoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. phytanate-CoA ligase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moreGolgi apparatus, mitochondrial matrix, cytosolOne of 5 routes

• prostaglandin H2Reach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. prostaglandin-endoperoxide synthase (arachidonate)

  Catalysed by ASAH1, PTGS1, PTGS2lysosome, extracellular space, cytosolOne of 3 routes

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

  Catalysed by ASAH1, ALOX5, ALOX15, ALOX12lysosome, extracellular space

• (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + O2 = (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate

  Catalysed by ASAH1, ALOX15B, ALOX15, ALOX12lysosome, extracellular space

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

  Catalysed by ASAH1, ALOX5lysosome, extracellular space

• 7,8-dihydrofolateReach 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. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)

  Catalysed by GBA1, GBA2, CH25H, MTO1

• leukotriene A4Reach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + O2 = leukotriene A4 + H2O

  Catalysed by ASAH1, ALOX5lysosome, extracellular space

• (13S)-hydroperoxy-(9Z,11E)-octadecadienoateReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. Linoleate:oxygen 13-oxidoreductase

  Catalysed by ASAH1, ALOX15B, ALOX15lysosome, extracellular space, cytosol

• 1-hexadecanoyl-sn-glycero-3-phosphateReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. 1-acylglycerol-3-phosphate pool formation and breakdown

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

• a long-chain fatty acidReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. dihydroxyacetone phosphate + an acyl-CoA = a 1-acylglycerone 3-phosphate + CoA
  3. a long chain fatty alcohol + a 1-acylglycerone 3-phosphate = a 1-O-alkylglycerone 3-phosphate + a long-chain fatty acid + H(+)

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

• (R)-2-hydroxyoctadecanoateReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. octadecanoate + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (R)-2-hydroxyoctadecanoate + 2 Fe(III)-[cytochrome b5] + H2O

  Catalysed by ASAH1, FA2Hlysosome, extracellular space

• ethanolamineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. Acyl-CoA:1-acyl-sn-glycero-3-phosphoethanolamine O-acyltransferase
  3. L-1-phosphatidylethanolamine:L-serine phosphatidyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 6 moreGolgi apparatus, cytosolOne of 4 routes

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol-5-phosphate)Reach 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) + ATP = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol-5-phosphate) + ADP + H(+)

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

• sphinganineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. sphingosine N-acyltransferase (sphinganine)
  3. an N-acylsphinganine + H2O = sphinganine + a fatty acid

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

• spermidineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 6 moreGolgi apparatus, mitochondrial matrix, cytosolOne of 3 routes

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

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 moreGolgi apparatus, mitochondrial matrix

• a 2-acyl-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. N-(acetyl)-sphing-4-enine + a 1,2-diacyl-sn-glycero-3-phosphocholine = 1-O-acyl-N-(acetyl)-sphing-4-enine + a 2-acyl-sn-glycero-3-phosphocholine

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 moreGolgi apparatusOne of 15 routes

• CDP-ethanolamineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. Acyl-CoA:1-alkenylglycerophosphoethanolamine O-acyltransferase
  3. ethanolaminephosphotransferase (1-alkenyl-2-acylglycerol)

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

• L-alanineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. amino-acid N-acetyltransferase (N-acetyl-L-alanine)

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

• L-asparagineReach 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-asparagine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 moreGolgi apparatus, mitochondrial matrix

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol-3-phosphate)Reach 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) + ATP = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol-3-phosphate) + ADP + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 9 moreGolgi apparatus

• L,L-cystathionineReach 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-cystathionine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 moreGolgi apparatus, mitochondrial matrix

• a 1-acyl-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. N-(acetyl)-sphing-4-enine + a 1,2-diacyl-sn-glycero-3-phosphocholine = 1-O-acyl-N-(acetyl)-sphing-4-enine + a 1-acyl-sn-glycero-3-phosphocholine

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 moreGolgi apparatusOne of 16 routes

• phosphoethanolamineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. sphing-4-enine + ATP = sphing-4-enine 1-phosphate + ADP + H(+)
  3. sphing-4-enine 1-phosphate = (2E)-hexadecenal + phosphoethanolamine

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

• 3-oxosphinganineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. Palmitoyl-CoA:L-serine C-palmitoyltransferase (decarboxylating)

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

• 1-hexadecanoylglycerone 3-phosphateReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. glyceronephosphate O-acyltransferase (DHAP)

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

• a CDP-1,2-diacyl-sn-glycerolReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. a 1-acyl-sn-glycero-3-phosphate + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phosphate + CoA
  3. a 1,2-diacyl-sn-glycero-3-phosphate + CTP + H(+) = a CDP-1,2-diacyl-sn-glycerol + diphosphate

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 16 moreGolgi apparatusOne of 3 routes

• alpha-D-galactose 1-phosphateReach from here →
  1. UDP-alpha-D-galactose:N-acylsphingosine D-galactosyltransferase
  2. D-galactosyl-N-acylsphingosine galactohydrolase
  3. galactokinase (galactose)

  Catalysed by UGT8, GALC, GALK1, GALK2cytosol, lysosomeOne of 2 routes

• 1D-myo-inositol 1-phosphateReach 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) + H2O = 1D-myo-inositol 1-phosphate + a 1,2-diacyl-sn-glycerol + H(+)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 moreGolgi apparatus

• (3Z)-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-5-enoyl-CoA)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 moreGolgi apparatus, mitochondrial matrixOne of 4 routes

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 6 moreGolgi apparatus, mitochondrial matrix, endoplasmic reticulumOne of 3 routes

• CDP-cholineReach from here →
  1. ceramide:phosphatidylcholine cholinephosphotransferase
  2. a sphingomyelin + H2O = phosphocholine + an N-acylsphing-4-enine + H(+)
  3. phosphocholine + CTP + H(+) = CDP-choline + diphosphate

  Catalysed by SGMS1, SGMS2, SMPD2, SMPD1 and 5 moreGolgi apparatusOne of 2 routes

• deoxycholoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 6 moreGolgi apparatus, mitochondrial matrix, cytosolOne of 4 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + H2O = (5Z,8Z,11Z,14Z)-eicosatetraenoate + glycine

  Catalysed by ASAH1, FAAH, PM20D1lysosome, extracellular space

• 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholineReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. (9Z,12Z)-octadecadienoyl-CoA + a 1-acyl-sn-glycero-3-phosphocholine = 1-acyl-2-(9Z,12Z)-octadecadienoyl-sn-glycero-3-phosphocholine + CoA

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

• a 2,3-dehydroacyl-CoAReach 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 2,3-saturated acyl-CoA + A = a 2,3-dehydroacyl-CoA + AH2

  Catalysed by GBA1, GBA2, CH25H, ACAD10

• a ganglioside GM3 (d18:1(4E))Reach from here →
  1. UDP-glucose:N-acylsphingosine D-glucosyltransferase
  2. glucosylceramide pool to LacCer pool conversion
  3. ST3 beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)

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

• (9S)-hydroperoxy-(10E,12Z)-octadecadienoateReach from here →
  1. N-Acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. linoleate:oxygen 9-oxidoreductase

  Catalysed by ASAH1lysosome, extracellular space, cytosol

• (2E)-tetradecenoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA pool formation and breakdown
  3. trans-tetradec-2-enoyl-CoA reductase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moreGolgi apparatus, cytosol, mitochondrial matrixOne of 5 routes

• decanoyl-CoAReach from here →
  1. acyl-CoA:sphingosine N-acyltransferase
  2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 moreGolgi apparatus, mitochondrial matrix, cytosolOne of 9 routes