D-glucosyl-N-acylsphingosine

Three steps

• an N-acylsphinganineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphingosine N-acyltransferase (sphinganine)

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 9 morecytosol, Golgi apparatusOne of 3 routes

• an N-acyl-(4R)-4-hydroxysphinganineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphocholine = an N-acyl-(4R)-4-hydroxysphinganine-phosphocholine + a 1,2-diacyl-sn-glycerol

  Catalysed by GBA1, GBA3, GBA2, SGMS1 and 1 morecytosol, Golgi apparatus

• eicosanoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, GBA2, ASAH1cytosol, lysosome, extracellular spaceOne of 12 routes

• (9Z)-hexadecenoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, GBA2, ASAH1cytosol, lysosome, extracellular spaceOne of 12 routes

• (15Z)-tetracosenoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, GBA2, ASAH1cytosol, lysosome, extracellular spaceOne of 12 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, GBA2, ASAH1cytosol, lysosome, extracellular spaceOne of 12 routes

• (11Z)-eicosenoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, GBA2, ASAH1cytosol, lysosome, extracellular spaceOne of 12 routes

• (24S)-hydroxycholesterolReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  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 = (24S)-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by GBA1, GBA3, GBA2, CYP46A1cytosol

• N-acetylneuraminateReach from here →
  1. glucosylceramide pool to LacCer pool conversion
  2. ST3 beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)
  3. a ganglioside GM3 (d18:1(4E)) + H2O = a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) + N-acetylneuraminate

  Catalysed by ALG1L2, B4GALT5, B3GALT2, B4GALT3 and 34 moreendoplasmic reticulum, cytosol

• a fatty aldehydeReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. an N-acylsphing-4-enine + H2O = sphing-4-enine + a fatty acid
  3. a fatty aldehyde + NAD(+) + H2O = a fatty acid + NADH + 2 H(+)

  Catalysed by GBA1, GBA3, GBA2, NAAA and 6 morecytosol

• cholesteryl hexadecanoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. cholesterol + hexadecanoyl-CoA = cholesteryl hexadecanoate + CoA

  Catalysed by GBA1, GBA3, GBA2, SOAT1cytosol

• a 1-O-alkyl-2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-alkyl-sn-glycero-3-phosphocholine O-acyltransferase

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 7 morecytosol, Golgi apparatus

• sphingosine 1-phosphateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. ATP:sphingosine 1-phosphotransferase

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 6 morecytosol, Golgi apparatusOne of 2 routes

• D-galactoseReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. an N-acylsphing-4-enine + UDP-alpha-D-galactose = a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + UDP + H(+)
  3. a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + H2O = D-galactose + an N-acylsphing-4-enine

  Catalysed by GBA1, GBA3, GBA2, UGT8 and 1 morecytosolOne of 2 routes

• cholesteryl (9Z,12Z)-octadecadienoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  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)-octadecadienoyl-CoA + cholesterol = cholesteryl (9Z,12Z)-octadecadienoate + CoA

  Catalysed by GBA1, GBA3, GBA2, SOAT1cytosolOne of 2 routes

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

  Catalysed by GBA1, GBA3, GBA2, CH25HcytosolOne of 3 routes

• N-acetyl-beta-neuraminic acidReach from here →
  1. glucosylceramide pool to LacCer pool conversion
  2. ST3 beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)
  3. exo-alpha-sialidase (GM3)

  Catalysed by ALG1L2, B4GALT5, B3GALT2, B4GALT3 and 36 moreendoplasmic reticulum, cytosolOne of 4 routes

• N-octanoylsphing-4-enineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. beta-D-glucosyl-N-octanoylsphing-4E-enine + cholesterol = N-octanoylsphing-4-enine + cholesteryl 3-beta-D-glucoside

  Catalysed by GBA1, GBA3, GBA2cytosol

• cholesteryl (9Z-octadecenoate)Reach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. cholesterol + (9Z)-octadecenoyl-CoA = cholesteryl (9Z-octadecenoate) + CoA

  Catalysed by GBA1, GBA3, GBA2, SOAT2 and 1 morecytosolOne of 2 routes

• N-(acetyl)-sphing-4-enineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  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, GBA2, ASAH1 and 4 morecytosol, Golgi apparatusOne of 2 routes

• a 1,3-diacylglycerolReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acylglycerol + an acyl-CoA = a 1,3-diacylglycerol + CoA

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 5 morecytosol, Golgi apparatus

• keto-D-fructoseReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. Aldehyde Reductase
  3. keto-D-fructose + NADH + H(+) = D-sorbitol + NAD(+)

  Catalysed by GBA1, GBA3, GBA2, SORDcytosol

• a 1,2-diacylglycerolReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acylglycerol + an acyl-CoA = a 1,2-diacylglycerol + CoA

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 8 morecytosol, Golgi apparatusOne of 2 routes

• (24R)-hydroxycholesterolReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  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 = (24R)-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by GBA1, GBA3, GBA2, CYP3A4cytosol

• a 1,2-diacyl-sn-glycero-3-phospho-L-serineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phospho-L-serine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-L-serine + CoA

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 6 morecytosol, Golgi apparatus

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 3 morecytosol, Golgi apparatus

• dodecanoyl-CoAReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 3 morecytosol, Golgi apparatus

• eicosanoyl-CoAReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 3 morecytosol, Golgi apparatus

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

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 3 morecytosol, Golgi apparatus

• tetradecanoyl-CoAReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 3 morecytosol, Golgi apparatus

• octadecanoyl-CoAReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA pool formation and breakdown

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 3 morecytosol, Golgi apparatus

• a 1-acylglycerone 3-phosphateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. dihydroxyacetone phosphate + an acyl-CoA = a 1-acylglycerone 3-phosphate + CoA

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 4 morecytosol, Golgi apparatus

• dCMPReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. dATP:D-glucose 6-phosphotransferase
  3. cytidine/uridine monophosphate kinase (dADP)

  Catalysed by GBA1, GBA3, GBA2, HK1 and 4 morecytosolOne of 2 routes

• a 1,2-diacyl-sn-glycero-3-phosphocholineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 2-acyl-sn-glycero-3-phosphocholine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 4 morecytosol, Golgi apparatusOne of 9 routes

• an N-acylglycineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 6 morecytosol, Golgi apparatus

• dGMPReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. dATP:D-glucose 6-phosphotransferase
  3. dADP to dATP conversion

  Catalysed by GBA1, GBA3, GBA2, HK1 and 3 morecytosol, mitochondrial matrix

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol)Reach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 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

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 5 morecytosol, Golgi apparatus

• 6-phospho-D-glucono-1,5-lactoneReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. ATP:D-glucose 6-phosphotransferase
  3. D-glucose-6-phosphate:NADP+ 1-oxidoreductase

  Catalysed by GBA1, GBA3, GBA2, HK1 and 5 morecytosol, endoplasmic reticulumOne of 6 routes

• a 1-acyl-sn-glycero-3-phosphateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sn-glycerol 3-phosphate + an acyl-CoA = a 1-acyl-sn-glycero-3-phosphate + CoA

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 7 morecytosol, Golgi apparatus

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol 4-phosphate)Reach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol 4-phosphate)(out) + cholesterol(in) = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol 4-phosphate)(in) + cholesterol(out)

  Catalysed by GBA1, GBA3, GBA2, OSBPcytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• alpha-D-galactose 1-phosphateReach from here →
  1. glucosylceramide pool to LacCer pool conversion
  2. beta-D-Galactosyl-1,4-beta-D-glucosylceramide galactohydrolase
  3. galactokinase (galactose)

  Catalysed by ALG1L2, B4GALT5, B3GALT2, B4GALT3 and 37 moreendoplasmic reticulum, lysosome, cytosolOne of 2 routes

• 1D-myo-inositol 3-phosphateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. ATP:D-glucose 6-phosphotransferase
  3. 1D-myo-inositol-3-phosphate lyase (isomerizing)

  Catalysed by GBA1, GBA3, GBA2, HK1 and 5 morecytosolOne of 2 routes

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol-4,5-bisphosphate)Reach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + 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, GBA2, OSBP2 and 1 morecytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. N-Acylsphingosine amidohydrolase
  3. (10Z)-heptadecenoic acid formation

  Catalysed by GBA1, GBA3, GBA2, ASAH1cytosol, lysosome, extracellular spaceOne of 12 routes

• a 1,2-diacyl-sn-glycero-3-phosphateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  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, GBA2, ASAH1 and 16 morecytosol, Golgi apparatusOne of 2 routes

• D-mannose 6-phosphateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. D-glucose + ADP = D-glucose 6-phosphate + AMP + H(+)
  3. D-glucose 6-phosphate = D-mannose 6-phosphate

  Catalysed by GBA1, GBA3, GBA2, ADPGK and 1 morecytosol

• sphing-4-enine 1-phosphateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + ATP = sphing-4-enine 1-phosphate + ADP + H(+)

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 5 morecytosol, Golgi apparatus

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

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 3 morecytosol, Golgi apparatus

• a cardiolipinReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  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, GBA2, ASAH1 and 4 morecytosol, Golgi apparatus

• an all-trans-retinyl esterReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. all-trans-retinol + an acyl-CoA = an all-trans-retinyl ester + CoA

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 7 morecytosol, Golgi apparatusOne of 2 routes