CHEBI:12947

Three steps

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

  Catalysed by GALC, HK1, HK2, HK3 and 2 morelysosome, cytosolOne of 2 routes

• a 1,2-diacyl-sn-glycero-3-phosphocholineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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 GALC, ASAH1, ACER2, ACER1 and 2 morelysosome, Golgi apparatusOne of 9 routes

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

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 4 morelysosome, Golgi apparatus

• dGMPReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. dATP:D-glucose 6-phosphotransferase
  3. dADP to dATP conversion

  Catalysed by GALC, HK1, HK2, HK3 and 1 morelysosome, cytosol, mitochondrial matrix

• N-sulfo-D-glucosamineReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 1 morelysosome

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

• dehydroepiandrosterone 3-sulfateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 1 morelysosome

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

  Catalysed by GALC, HK1, GCK, HK2 and 3 morelysosome, cytosol, endoplasmic reticulumOne of 6 routes

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

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

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol 4-phosphate)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. 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 GALC, GBA1, GBA2, OSBPlysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• adenosine 5'-phosphosulfateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. ATP:sulfate adenylyltransferase

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 2 morelysosome, cytosolOne of 4 routes

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

  Catalysed by GALC, HK1, GCK, HK2 and 3 morelysosome, cytosolOne of 2 routes

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol-4,5-bisphosphate)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. 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 GALC, GBA1, GBA2, OSBP2 and 1 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoateReach 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

• alpha-D-glucose 1-phosphateReach from here →
  1. D-galactosyl-N-acylsphingosine galactohydrolase
  2. galactokinase (galactose)
  3. alpha-D-galactose 1-phosphate + UDP-alpha-D-glucose = alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose

  Catalysed by GALC, GALK1, GALK2, GALTlysosome, cytosol

• a 1,2-diacyl-sn-glycero-3-phosphateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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 GALC, ASAH1, ACER2, ACER1 and 14 morelysosome, Golgi apparatusOne of 2 routes

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

  Catalysed by GALC, ADPGK, GPIlysosome

• estrone 3-sulfateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. estrone 3-sulfate + H2O = estrone + sulfate + H(+)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 1 morelysosome

• a ganglioside GM3 (d18:1(4E))Reach from here →
  1. D-galactosyl-N-acylsphingosine galactohydrolase
  2. beta-galactosidase (galactose)
  3. ST3 beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by GALC, LCT, GLB1, GLB1L and 1 morelysosome, extracellular space, cytosolOne of 3 routes

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

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

• (9Z)-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

• a cardiolipinReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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 GALC, ASAH1, ACER2, ACER1 and 2 morelysosome, Golgi apparatus

• (5-hydroxyindol-3-yl)acetateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. Transport of 5-Hydroxyindoleacetate

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 1 morelysosome, extracellular space, cytosolOne of 2 routes

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

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 5 morelysosome, Golgi apparatusOne of 2 routes

• dTMPReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. dATP:D-glucose 6-phosphotransferase
  3. deoxythymidylate kinase (dADP)

  Catalysed by GALC, HK1, HK2, HK3 and 2 morelysosome, cytosol, mitochondrial matrixOne of 2 routes

• CHEBI:63544Reach 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

• 1-O-hexadecyl-sn-glycero-3-phosphocholineReach 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 apparatus

• a 1,2-diacyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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 GALC, ASAH1, ACER2, ACER1 and 5 morelysosome, Golgi apparatusOne of 2 routes

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

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 7 morelysosome, Golgi apparatusOne of 2 routes

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

• hexacosanoyl-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

• tetracosanoyl-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

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

• UDP-alpha-D-galactoseReach from here →
  1. D-galactosyl-N-acylsphingosine galactohydrolase
  2. galactokinase (galactose)
  3. alpha-D-galactose 1-phosphate + UDP-alpha-D-glucose = alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose

  Catalysed by GALC, GALK1, GALK2, GALTlysosome, cytosol

• N-hexadecanoylsphinganineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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 GALC, SGMS1, SGMS2lysosome, Golgi apparatus

• CHEBI:67119Reach from here →
  1. D-galactosyl-N-acylsphingosine galactohydrolase
  2. galactokinase (galactose)
  3. UDP-glucose:alpha-D-galactose-1-phosphate uridylyltransferase

  Catalysed by GALC, GALK1, GALK2, GALTlysosome, cytosolOne of 2 routes

• (22R)-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. 2 reduced [adrenodoxin] + cholesterol + O2 + 2 H(+) = (22R)-hydroxycholesterol + 2 oxidized [adrenodoxin] + H2O

  Catalysed by GALC, GBA1, GBA2, CYP11A1lysosomeOne of 2 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-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)-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

• 1,2-dihexadecanoyl-sn-glycero-3-phosphateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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 GALC, SGMS2, DGKAlysosome

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

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

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

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

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

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 5 morelysosome, Golgi apparatusOne of 3 routes

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

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 5 morelysosome, Golgi apparatusOne of 3 routes

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

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

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

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

• 1-hexadecanoyl-sn-glycero-3-phosphocholineReach 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-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-sn-glycero-3-phosphocholine + cholesterol = cholesteryl (5Z,8Z,11Z,14Z)-eicosatetraenoate + 1-hexadecanoyl-sn-glycero-3-phosphocholine

  Catalysed by GALC, GBA1, GBA2, LCATlysosomeOne of 9 routes

• 1,2-dihexadecanoyl-sn-glycero-3-phosphocholineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  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 GALC, SGMS1, SGMS2lysosome, Golgi apparatusOne of 2 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-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