CHEBI:12947

Three steps

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (13Z)-docosenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (11Z)-eicosenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (15Z)-tetracosenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (17Z)-hexacosenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• heptadecanoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (9Z)-heptadecenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• N-(15Z-tetracosenoyl)-sphing-4-enineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. N-(15Z-tetracosenoyl)-sphing-4-enine + H2O = (15Z)-tetracosenoate + sphing-4-enine

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatusOne of 2 routes

• N-(tetracosenoyl)-sphing-4-enineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. tetracosenoyl-CoA + sphing-4-enine = N-(tetracosenoyl)-sphing-4-enine + CoA + H(+)

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 2 morelysosome, Golgi apparatus

• nonadecanoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• a 2,3-diacyl-sn-glycerolReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acylglycerol + an acyl-CoA = a 2,3-diacyl-sn-glycerol + CoA

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 3 morelysosome, Golgi apparatus

• 1-O-alkyl-3-acylglycerolReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-O-alkylglycerol + an acyl-CoA = 1-O-alkyl-3-acylglycerol + CoA

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 3 morelysosome, Golgi apparatus

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (25R)-cholest-5-ene-3beta,26-diolReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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(+) = (25R)-cholest-5-ene-3beta,26-diol + 2 oxidized [adrenodoxin] + H2O

  Catalysed by GALC, GBA1, GBA2, CYP27A1lysosome

• 1-octadecanoyl-2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phospho-1D-myo-inositol 4,5-bisphosphateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. 1-octadecanoyl-2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phospho-1D-myo-inositol 4,5-bisphosphate(out) + cholesterol(in) = 1-octadecanoyl-2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phospho-1D-myo-inositol 4,5-bisphosphate(in) + cholesterol(out)

  Catalysed by GALC, GBA1, GBA2, OSBPL2lysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• (11Z,14Z)-eicosadienoateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GALC, ASAH1lysosome, extracellular spaceOne of 12 routes

• (17Z)-hexacosenoateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GALC, ASAH1lysosome, extracellular spaceOne of 12 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GALC, ASAH1lysosome, extracellular spaceOne of 12 routes

• (7Z,10Z,13Z,16Z)-docosatetraenoateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GALC, ASAH1lysosome, extracellular spaceOne of 12 routes

• a 1,3-diacyl-sn-glycerolReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 3-acyl-sn-glycerol + an acyl-CoA = a 1,3-diacyl-sn-glycerol + CoA

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 2 morelysosome, Golgi apparatusOne of 3 routes

• a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-O-(1Z-alkenyl)-sn-glycero-3-phosphocholine + an acyl-CoA = a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 3 morelysosome, Golgi apparatus

• a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-O-(1Z-alkenyl)-sn-glycero-3-phosphoethanolamine + an acyl-CoA = a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 2 morelysosome, Golgi apparatus

• a fatty acyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. an N-acylsphing-4-enine + H2O = sphing-4-enine + a fatty acid
  3. a fatty acid + ATP + CoA = a fatty acyl-CoA + AMP + diphosphate

  Catalysed by GALC, NAAA, ASAH1, ACER2 and 4 morelysosome

• N-(9Z-octadecenoyl)-sphing-4-enineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. N-(9Z-octadecenoyl)-sphing-4-enine + H2O = sphing-4-enine + (9Z)-octadecenoate

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 2 morelysosome, Golgi apparatusOne of 3 routes

• (5Z,8Z,11Z)-eicosatrienoateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GALC, ASAH1lysosome, extracellular spaceOne of 12 routes

• a 1-(9Z-octadecenoyl)-2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. 1-(9Z-octadecenoyl)-2-acyl-sn-3-glycerol + a sphingomyelin = a 1-(9Z-octadecenoyl)-2-acyl-sn-glycero-3-phosphocholine + an N-acylsphing-4-enine

  Catalysed by GALC, SGMS1, SGMS2lysosome, Golgi apparatusOne of 2 routes

• beta-D-3-sulfogalactosyl-(1->4)-beta-D-glucosyl-(1<->1')-N-acylsphing-4-enineReach from here →
  1. D-galactosyl-N-acylsphingosine galactohydrolase
  2. beta-galactosidase (galactose)
  3. a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) + 3'-phosphoadenylyl sulfate = beta-D-3-sulfogalactosyl-(1->4)-beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by GALC, LCT, GLB1, GLB1L and 1 morelysosome, extracellular space

• a ganglioside GM4 (d18:1(4E))Reach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. an N-acyl-1-beta-D-(3-O-sulfo)-galactosyl-sphing-4-enine + H2O = a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + sulfate + H(+)
  3. a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + CMP-N-acetyl-beta-neuraminate = a ganglioside GM4 (d18:1(4E)) + CMP + H(+)

  Catalysed by GAL3ST1, ARSA, ST3GAL5lysosome

• tricosanoateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GALC, ASAH1lysosome, extracellular spaceOne of 12 routes

• cholesteryl octadecanoateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. octadecanoyl-CoA + cholesterol = cholesteryl octadecanoate + CoA

  Catalysed by GALC, GBA1, GBA2, SOAT1lysosome

• cholesteryl (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + cholesterol = cholesteryl (5Z,8Z,11Z,14Z)-eicosatetraenoate + CoA

  Catalysed by GALC, GBA1, GBA2, SOAT2 and 1 morelysosomeOne of 2 routes

• sphinga-4E,14Z-dienineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = sphinga-4E,14Z-dienine + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 2 morelysosome, Golgi apparatus

• beta-D-glucosyl-(1<->1)-sphing-4-enineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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 + H2O = beta-D-glucosyl-(1<->1)-sphing-4-enine + a fatty acid

  Catalysed by GALC, UGCG, ASAH1lysosome

• cholesteryl (9Z)-hexadecenoateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. (9Z)-hexadecenoyl-CoA + cholesterol = cholesteryl (9Z)-hexadecenoate + CoA

  Catalysed by GALC, GBA1, GBA2, SOAT1lysosome

• (9Z,12Z,15Z-octadecatrienoyl)-cholesterolReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. (9Z,12Z,15Z)-octadecatrienoyl-CoA + cholesterol = (9Z,12Z,15Z-octadecatrienoyl)-cholesterol + CoA

  Catalysed by GALC, GBA1, GBA2, SOAT2lysosomeOne of 2 routes

• cholesteryl (8Z,11Z,14Z)-eicosatrienoateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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-(8Z,11Z,14Z-eicosatrienoyl)-sn-glycero-3-phosphocholine + cholesterol = cholesteryl (8Z,11Z,14Z)-eicosatrienoate + 1-hexadecanoyl-sn-glycero-3-phosphocholine

  Catalysed by GALC, GBA1, GBA2, LCATlysosome

• (5Z)-tetradecenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol

• (5Z,8Z,11Z,14Z,17Z-eicosapentaenoyl)-cholesterolReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + cholesterol = (5Z,8Z,11Z,14Z,17Z-eicosapentaenoyl)-cholesterol + CoA

  Catalysed by GALC, GBA1, GBA2, SOAT2lysosomeOne of 2 routes

• 4beta-hydroxycholesterolReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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 = 4beta-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by GALC, GBA1, GBA2, CYP3A4lysosome

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

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 3 morelysosome, Golgi apparatus

• (7Z)-hexadecenoyl-CoAReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 1 morelysosome, Golgi apparatus, cytosol