CHEBI:12947

Three steps

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

• beta-D-fructoseReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. Aldehyde Reductase
  3. D-Glucitol:NAD+ 2-oxidoreductase

  Catalysed by GALC, SORDlysosome, cytosol

• a dicarboxylateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 2 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• chlorine(.)Reach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. transport of chloride and sulfate (cytosol to extracellular)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 1 morelysosome, extracellular space, cytosol

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

  Catalysed by GALC, SGMS1, SGMS2, SMPD2 and 4 morelysosome, Golgi apparatus

• D-aspartateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. D-aspartate(in) + sulfate(out) = D-aspartate(out) + sulfate(in)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 2 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-aspartateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 2 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• oxalateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. oxalate(in) + sulfate(out) = oxalate(out) + sulfate(in)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 5 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• maleateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. maleate(in) + sulfate(out) = maleate(out) + sulfate(in)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 2 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• malonic acidReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. transport of malonate and sulfate (cytosol to mitochondria)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 1 morelysosome, mitochondrial matrix, cytosolOne of 2 routes

• tetradecanoateReach 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

• (9Z)-octadecenoateReach 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

• (2R,3R)-tartrateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. (2R,3R)-tartrate(in) + sulfate(out) = (2R,3R)-tartrate(out) + sulfate(in)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 2 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• (S,S)-tartrateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. (S,S)-tartrate(in) + sulfate(out) = (S,S)-tartrate(out) + sulfate(in)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 2 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3R)-citramalateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. (3R)-citramalate(in) + sulfate(out) = (3R)-citramalate(out) + sulfate(in)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 2 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3S)-citramalateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. (3S)-citramalate(in) + sulfate(out) = (3S)-citramalate(out) + sulfate(in)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 2 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• hexacosanoateReach 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

• tetracosanoateReach 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

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

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 7 morelysosome, Golgi apparatus, cytosolOne of 3 routes

• an N-acyl-(4R)-4-hydroxysphinganineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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 GALC, SGMS1, SGMS2lysosome, Golgi apparatus

• eicosanoateReach 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

• (9Z)-hexadecenoateReach 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

• (15Z)-tetracosenoateReach 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

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach 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

• (11Z)-eicosenoateReach 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

• thiosulfateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. thiosulfate(in) + sulfate(out) = thiosulfate(out) + sulfate(in)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 4 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• (24S)-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 = (24S)-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by GALC, GBA1, GBA2, CYP46A1lysosome

• a fatty aldehydeReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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 GALC, NAAA, ASAH1, ACER2 and 4 morelysosome

• cholesteryl hexadecanoateReach 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 + hexadecanoyl-CoA = cholesteryl hexadecanoate + CoA

  Catalysed by GALC, GBA1, GBA2, SOAT1lysosome

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

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 5 morelysosome, Golgi apparatus, cytosol

• sphingosine 1-phosphateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. ATP:sphingosine 1-phosphotransferase

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 4 morelysosome, Golgi apparatus, cytosolOne of 2 routes

• D-galactoseReach 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 + H2O = D-galactose + an N-acylsphing-4-enine

  Catalysed by GAL3ST1, ARSA, GBA1, GALC and 2 morelysosomeOne of 3 routes

• cholesteryl (9Z,12Z)-octadecadienoateReach 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)-octadecadienoyl-CoA + cholesterol = cholesteryl (9Z,12Z)-octadecadienoate + CoA

  Catalysed by GALC, GBA1, GBA2, SOAT1lysosomeOne of 2 routes

• 25-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 + AH2 + O2 = 25-hydroxycholesterol + A + H2O

  Catalysed by GALC, GBA1, GBA2, CH25HlysosomeOne of 3 routes

• N-octanoylsphing-4-enineReach 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. beta-D-glucosyl-N-octanoylsphing-4E-enine + cholesterol = N-octanoylsphing-4-enine + cholesteryl 3-beta-D-glucoside

  Catalysed by GALC, GBA1, GBA2lysosome

• cholesteryl (9Z-octadecenoate)Reach 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 + (9Z)-octadecenoyl-CoA = cholesteryl (9Z-octadecenoate) + CoA

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

• N-(acetyl)-sphing-4-enineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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 GALC, ASAH1, ACER2, ACER1 and 2 morelysosome, Golgi apparatusOne of 2 routes

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

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

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

  Catalysed by GALC, SORDlysosome, cytosol

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

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 6 morelysosome, Golgi apparatusOne of 2 routes

• (24R)-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 = (24R)-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by GALC, GBA1, GBA2, CYP3A4lysosome

• an isogloboside iGb3Cer (d18:1(4E))Reach 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)) + UDP-alpha-D-galactose = an isogloboside iGb3Cer (d18:1(4E)) + UDP + H(+)

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

• a 1,2-diacyl-sn-glycero-3-phospho-L-serineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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 GALC, ASAH1, ACER2, ACER1 and 4 morelysosome, Golgi apparatus

• (6Z,9Z,12Z)-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

• dodecanoyl-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

• eicosanoyl-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)-octadecadienoyl-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

• tetradecanoyl-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

• octadecanoyl-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 1-acylglycerone 3-phosphateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. dihydroxyacetone phosphate + an acyl-CoA = a 1-acylglycerone 3-phosphate + CoA

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