D-glucosyl-N-acylsphingosine

Three steps

• thioredoxin dithiolReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. dATP:D-glucose 6-phosphotransferase
  3. 2-Deoxyuridine 5-Diphosphate:Oxidized-Thioredoxin 2-Oxidoreductase

  Catalysed by GBA1, GBA3, GBA2, HK1 and 8 morecytosol, mitochondrial matrixOne of 2 routes

• phosphatidylethanolamineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-acyl-sn-glycero-3-phosphoethanolamine O-acyltransferase

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

• CHEBI:16039Reach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. ITP:D-glucose 6-phosphotransferase
  3. ATP:IDP phosphotransferase

  Catalysed by GBA1, GBA3, GBA2, HK1 and 8 morecytosolOne of 3 routes

• 1,2-diacyl-sn-glycero-3-phosphocholine(1+)Reach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-acyl-sn-glycero-3-phosphocholine O-acyltransferase

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

• CHEBI:16284Reach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. dATP:D-glucose 6-phosphotransferase
  3. ATP:dADP phosphotransferase

  Catalysed by GBA1, GBA3, GBA2, HK1 and 8 morecytosolOne of 16 routes

• phosphatidic acidReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:1-acyl-sn-glycerol-3-phosphate 2-O-acyltransferase

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 12 morecytosol, Golgi apparatus, mitochondrial matrixOne of 9 routes

• CHEBI:16436Reach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. ceramide cholinephosphotransferase (CDP-choline)

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

• alpha,alpha-trehaloseReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. D-glucose 1-epimerase
  3. alpha,alpha-trehalose + H2O = alpha-D-glucose + beta-D-glucose

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

• pregnenoloneReach 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. 6 reduced [adrenodoxin] + cholesterol + 3 O2 + 6 H(+) = 4-methylpentanal + pregnenolone + 6 oxidized [adrenodoxin] + 4 H2O

  Catalysed by GBA1, GBA3, GBA2, CYP11A1cytosol

• galactitolReach from here →
  1. glucosylceramide pool to LacCer pool conversion
  2. beta-D-Galactosyl-1,4-beta-D-glucosylceramide galactohydrolase
  3. galactitol:NADP+ 1-oxidoreductase

  Catalysed by ALG1L2, B4GALT5, B3GALT2, B4GALT3 and 36 moreendoplasmic reticulum, lysosome, cytosol

• psychosineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase

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

• 1-acyl-sn-glycerol 3-phosphateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase

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

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

  Catalysed by GBA1, GBA3, GBA2, ASAH1 and 5 morecytosol, Golgi apparatusOne of 2 routes

• a neolactoside nLc4Cer(d18:1(4E))Reach from here →
  1. glucosylceramide pool to LacCer pool conversion
  2. UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase (LacCer pool)
  3. beta-1,4-galactosyltransferase (lc3Cer)

  Catalysed by ALG1L2, B4GALT5, B3GALT2, B4GALT3 and 31 moreendoplasmic reticulum, cytosolOne of 2 routes

• CHEBI:17202Reach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. ITP:D-glucose 6-phosphotransferase
  3. IDP phosphohydrolase

  Catalysed by GBA1, GBA3, GBA2, HK1 and 11 morecytosol, extracellular space

• a beta-D-Gal-(1->3)-beta-D-GlcNAc-(1->3)-beta-D-Gal-(1->4)-beta-D-Glc-(1<->1')-Cer(d18:1(4E))Reach from here →
  1. glucosylceramide pool to LacCer pool conversion
  2. UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase (LacCer pool)
  3. N-acetyl-beta-D-glucosaminide beta-(1,3)-galactosyltransferase (lc3Cer)

  Catalysed by ALG1L2, B4GALT5, B3GALT2, B4GALT3 and 31 moreendoplasmic reticulum, cytosolOne of 3 routes

• linoleic acidReach 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 2-acylglycerolReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. a 1,2-diacyl-sn-glycerol + H2O = a 2-acylglycerol + a fatty acid + H(+)

  Catalysed by GBA1, GBA3, GBA2, SGMS1 and 4 morecytosol, Golgi apparatus

• a monoacylglycerolReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. 1,2-diacyl-sn-glycerol acylhydrolase

  Catalysed by GBA1, GBA3, GBA2, SGMS1 and 16 morecytosol, Golgi apparatus, extracellular spaceOne of 9 routes

• 1-(alk-1-enyl)-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-alkenylglycerophosphoethanolamine O-acyltransferase

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

• cholesteryl 3-beta-D-glucosideReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  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 + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine

  Catalysed by GBA1, GBA3, GBA2, UGCGcytosolOne of 8 routes

• 7alpha-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 = 7alpha-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by GBA1, GBA3, GBA2, CYP7A1cytosol

• 26-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 = 26-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by GBA1, GBA3, GBA2, CYP3A4cytosol

• a triacylglycerolReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase

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

• alpha-D-glucoseReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. D-glucose 1-epimerase
  3. alpha-D-glucose = beta-D-glucose

  Catalysed by GBA1, GBA3, GBA2, GALMcytosol

• 4-methylpentanalReach 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. 6 reduced [adrenodoxin] + cholesterol + 3 O2 + 6 H(+) = 4-methylpentanal + pregnenolone + 6 oxidized [adrenodoxin] + 4 H2O

  Catalysed by GBA1, GBA3, GBA2, CYP11A1cytosol

• dTDPReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. dATP:D-glucose 6-phosphotransferase
  3. nucleoside-diphosphate kinase (dADP)

  Catalysed by GBA1, GBA3, GBA2, HK1 and 9 morecytosolOne of 3 routes

• CHEBI:18132Reach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. Sphingomyelin cholinephosphohydrolase

  Catalysed by GBA1, GBA3, GBA2, SGMS1 and 6 morecytosol, Golgi apparatusOne of 4 routes

• a globoside Gb4Cer (d18:1(4E))Reach from here →
  1. glucosylceramide pool to LacCer pool conversion
  2. alpha 1,4-galactosyltransferase (P blood group) (LacCer pool)
  3. globotriaosylceramide 3-beta-N-acetylgalactosaminyltransferase (globotriaosylceramide)

  Catalysed by ALG1L2, B4GALT5, B3GALT2, B4GALT3 and 32 moreendoplasmic reticulum, cytosolOne of 4 routes

• dodecanoateReach 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

• D-gluconateReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. D-glucose + NAD(+) = D-glucono-1,5-lactone + NADH + H(+)
  3. D-glucono-1,5-lactone + H2O = D-gluconate + H(+)

  Catalysed by GBA1, GBA3, GBA2, H6PD and 1 morecytosol

• cholesteryl 3-beta-D-galactosideReach 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 + cholesterol = cholesteryl 3-beta-D-galactoside + an N-acylsphing-4-enine

  Catalysed by GBA1, GBA3, GBA2, UGT8cytosolOne of 3 routes

• cholesteryl 3-beta-D-xylosideReach 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 beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine

  Catalysed by GBA1, GBA3, GBA2cytosolOne of 3 routes

• a lysoGb3(d18:1(4E))Reach from here →
  1. glucosylceramide pool to LacCer pool conversion
  2. alpha 1,4-galactosyltransferase (P blood group) (LacCer pool)
  3. a globoside Gb3Cer (d18:1(4E)) + H2O = a lysoGb3(d18:1(4E)) + a fatty acid

  Catalysed by ALG1L2, B4GALT5, B3GALT2, B4GALT3 and 33 moreendoplasmic reticulum, cytosol

• (11Z)-docosenoyl-CoA(4-)Reach 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

• (7Z)-tetradecenoyl-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

• NAADPHReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. D-glucose + ADP = D-glucose 6-phosphate + AMP + H(+)
  3. NAADP(+) + D-glucose 6-phosphate = NAADPH + 6-phospho-D-glucono-1,5-lactone + H(+)

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

• docosanoateReach 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

• octadecanoateReach 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

• alpha-linolenic acidReach 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

• beta-D-galactoseReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. D-glucose + UDP-alpha-D-galactose = lactose + UDP + H(+)
  3. lactose + H2O = beta-D-galactose + D-glucose

  Catalysed by GBA1, GBA3, GBA2, B4GALT2 and 2 morecytosolOne of 3 routes

• a ganglioside GA1 (d18:1(4E))Reach from here →
  1. glucosylceramide pool to LacCer pool conversion
  2. beta-1,4-N-acetyl-galactosaminyltransferase (LacCer pool)
  3. a ganglioside GA2 (d18:1(4E)) + UDP-alpha-D-galactose = a ganglioside GA1 (d18:1(4E)) + UDP + H(+)

  Catalysed by ALG1L2, B4GALT5, B3GALT2, B4GALT3 and 33 moreendoplasmic reticulum, cytosol

• elaidic acidReach 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 apparatusOne of 13 routes

• all-cis-docosa-4,7,10,13,16,19-hexaenoic acidReach 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

• beta-D-fructoseReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. Aldehyde Reductase
  3. D-Glucitol:NAD+ 2-oxidoreductase

  Catalysed by GBA1, GBA3, GBA2, SORDcytosol

• phosphocholineReach from here →
  1. D-Glucosyl-N-acylsphingosine glucohydrolase
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. a sphingomyelin + H2O = phosphocholine + an N-acylsphing-4-enine + H(+)

  Catalysed by GBA1, GBA3, GBA2, SGMS1 and 6 morecytosol, Golgi apparatus

• tetradecanoateReach 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)-octadecenoateReach 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

• hexacosanoateReach 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

• tetracosanoateReach 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