a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine

Three steps

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol-4,5-bisphosphate)Reach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  3. 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)

  Catalysed by GBA1, GBA3, OSBP2, OSBPL2inner side of the membrane, outer side of the membraneOne of 2 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoateReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, ASAH1lysosome, extracellular spaceOne of 12 routes

• a 1,2-diacyl-sn-glycero-3-phosphateReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phosphate + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phosphate + CoA

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 15 moreGolgi apparatusOne of 2 routes

• sphing-4-enine 1-phosphateReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + ATP = sphing-4-enine 1-phosphate + ADP + H(+)

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 4 moreGolgi apparatus

• (9Z)-hexadecenoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• a cardiolipinReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1'-[1,2-diacyl-sn-glycero-3-phospho],3'-[1-acyl-sn-glycero-3-phospho]-glycerol + an acyl-CoA = a cardiolipin + CoA

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 3 moreGolgi apparatus

• an all-trans-retinyl esterReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. all-trans-retinol + an acyl-CoA = an all-trans-retinyl ester + CoA

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 6 moreGolgi apparatusOne of 2 routes

• CHEBI:63544Reach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• 1-O-hexadecyl-sn-glycero-3-phosphocholineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 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(+)

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 3 moreGolgi apparatus

• a 1,2-diacyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phosphoethanolamine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 6 moreGolgi apparatusOne of 2 routes

• a triacyl-sn-glycerolReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + a 1,2-diacyl-sn-glycerol = a triacyl-sn-glycerol + CoA

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 8 moreGolgi apparatusOne of 2 routes

• a 1,2-diacyl-sn-glycero-3-phospho-(1'-sn-glycerol)Reach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-acyl-sn-glycero-3-phospho-(1'-sn-glycerol) + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-(1'-sn-glycerol) + CoA

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 5 moreGolgi apparatus

• hexacosanoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• tetracosanoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• docosanoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• N-hexadecanoyl-(4R)-hydroxysphinganineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. N-hexadecanoyl-(4R)-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphocholine = N-hexadecanoyl-(4R)-hydroxysphinganine-phosphocholine + a 1,2-diacyl-sn-glycerol

  Catalysed by GBA1, GBA3, SGMS1, SGMS2Golgi apparatus

• N-hexadecanoylsphinganineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. N-hexadecanoylsphinganine + a 1,2-diacyl-sn-glycero-3-phosphocholine = N-hexadecanoyl-sphinganine-1-phosphocholine + a 1,2-diacyl-sn-glycerol

  Catalysed by GBA1, GBA3, SGMS1, SGMS2Golgi apparatus

• (22R)-hydroxycholesterolReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  3. 2 reduced [adrenodoxin] + cholesterol + O2 + 2 H(+) = (22R)-hydroxycholesterol + 2 oxidized [adrenodoxin] + H2O

  Catalysed by GBA1, GBA3, CYP11A1One of 2 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (8Z,11Z,14Z)-eicosatrienoateReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, ASAH1lysosome, extracellular spaceOne of 12 routes

• 1,2-dihexadecanoyl-sn-glycero-3-phosphateReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. 1,2-dihexadecanoyl-sn-glycero-3-phosphocholine + an N-acylsphing-4-enine = 1,2-dihexadecanoyl-sn-glycerol + a sphingomyelin
  3. 1,2-dihexadecanoyl-sn-glycerol + ATP = 1,2-dihexadecanoyl-sn-glycero-3-phosphate + ADP + H(+)

  Catalysed by GBA1, GBA3, SGMS2, DGKA

• N-dodecanoylsphing-4-enineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  3. 1-(beta-D-galactosyl)-N-dodecanoylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-galactoside + N-dodecanoylsphing-4-enine

  Catalysed by GBA1, GBA3, GBA2One of 3 routes

• N-tetradecanoylsphing-4-enineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. N-tetradecanoylsphing-4-enine + H2O = sphing-4-enine + tetradecanoate

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus

• N-hexadecanoylsphing-4-enineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  3. beta-D-glucosyl-(1<->1')-N-hexadecanoylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + N-hexadecanoylsphing-4-enine

  Catalysed by GBA1, GBA3One of 3 routes

• N-octadecanoylsphing-4-enineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  3. beta-D-glucosyl-(1<->1)-N-octadecanoylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + N-octadecanoylsphing-4-enine

  Catalysed by GBA1, GBA3One of 3 routes

• N-eicosanoyl-sphing-4-enineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. eicosanoyl-CoA + sphing-4-enine = N-eicosanoyl-sphing-4-enine + CoA + H(+)

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 4 moreGolgi apparatus

• N-tetracosanoyl-sphing-4-enineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. tetracosanoyl-CoA + sphing-4-enine = N-tetracosanoyl-sphing-4-enine + CoA + H(+)

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 3 moreGolgi apparatus

• 1-hexadecanoyl-sn-glycero-3-phosphocholineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  3. 1-hexadecanoyl-2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphocholine + cholesterol = cholesteryl (5Z,8Z,11Z,14Z)-eicosatetraenoate + 1-hexadecanoyl-sn-glycero-3-phosphocholine

  Catalysed by GBA1, GBA3, LCATOne of 9 routes

• 1,2-dihexadecanoyl-sn-glycero-3-phosphocholineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. 1,2-dihexadecanoyl-sn-glycero-3-phosphocholine + an N-acylsphing-4-enine = 1,2-dihexadecanoyl-sn-glycerol + a sphingomyelin

  Catalysed by GBA1, GBA3, SGMS1, SGMS2Golgi apparatusOne of 2 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (13Z)-docosenoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (11Z)-eicosenoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (15Z)-tetracosenoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (17Z)-hexacosenoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• heptadecanoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (9Z)-heptadecenoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• N-(15Z-tetracosenoyl)-sphing-4-enineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  3. beta-D-glucosyl-(1<->1')-N-(15Z-tetracosenoyl)-sphing-4-enine + cholesterol = N-(15Z-tetracosenoyl)-sphing-4-enine + cholesteryl 3-beta-D-glucoside

  Catalysed by GBA1, GBA3One of 2 routes

• N-(tetracosenoyl)-sphing-4-enineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. tetracosenoyl-CoA + sphing-4-enine = N-(tetracosenoyl)-sphing-4-enine + CoA + H(+)

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 3 moreGolgi apparatus

• nonadecanoyl-CoAReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 2 moreGolgi apparatus, cytosol

• a 2,3-diacyl-sn-glycerolReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acylglycerol + an acyl-CoA = a 2,3-diacyl-sn-glycerol + CoA

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 4 moreGolgi apparatus

• an N-acyl-1-beta-D-(3-O-sulfo)-galactosyl-sphing-4-enineReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. UDP-alpha-D-galactose:N-acylsphingosine D-galactosyltransferase
  3. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase

  Catalysed by GBA1, GBA3, UGT8, GAL3ST1cytosol, lysosomeOne of 2 routes

• 1-O-alkyl-3-acylglycerolReach from here →
  1. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-O-alkylglycerol + an acyl-CoA = 1-O-alkyl-3-acylglycerol + CoA

  Catalysed by GBA1, GBA3, ASAH1, ACER2 and 4 moreGolgi apparatus