CHEBI:67119

Three steps

• N-tetradecanoylsphing-4-enineReach from here →
  1. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  2. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  3. N-tetradecanoylsphing-4-enine + H2O = sphing-4-enine + tetradecanoate

  Catalysed by ASAH1, ACER2, ASAH2cytosol

• N-hexadecanoylsphing-4-enineReach from here →
  1. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  2. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  3. sphing-4-enine + hexadecanoyl-CoA = N-hexadecanoylsphing-4-enine + CoA + H(+)

  Catalysed by CERS6, TLCD3B, CERS5, CERS2cytosolOne of 2 routes

• N-octadecanoylsphing-4-enineReach from here →
  1. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  2. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  3. sphing-4-enine + octadecanoyl-CoA = N-octadecanoylsphing-4-enine + CoA + H(+)

  Catalysed by CERS1, TLCD3B, CERS2, CERS4cytosolOne of 2 routes

• N-eicosanoyl-sphing-4-enineReach from here →
  1. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  2. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  3. eicosanoyl-CoA + sphing-4-enine = N-eicosanoyl-sphing-4-enine + CoA + H(+)

  Catalysed by TLCD3B, CERS2cytosol

• N-tetracosanoyl-sphing-4-enineReach from here →
  1. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  2. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  3. tetracosanoyl-CoA + sphing-4-enine = N-tetracosanoyl-sphing-4-enine + CoA + H(+)

  Catalysed by CERS2cytosol

• an N-acylsphing-4-enine 1-phosphoethanolamineReach from here →
  1. UDP-alpha-D-galactose:N-acylsphingosine D-galactosyltransferase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. an N-acylsphing-4-enine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acylsphing-4-enine 1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol

  Catalysed by UGT8, GALC, SGMS1, SGMS2 and 1 morecytosol, lysosome

• N-(15Z-tetracosenoyl)-sphing-4-enineReach from here →
  1. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  2. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  3. N-(15Z-tetracosenoyl)-sphing-4-enine + H2O = (15Z)-tetracosenoate + sphing-4-enine

  Catalysed by ACER2, ACER1, ASAH2cytosol

• N-(tetracosenoyl)-sphing-4-enineReach from here →
  1. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  2. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  3. tetracosenoyl-CoA + sphing-4-enine = N-(tetracosenoyl)-sphing-4-enine + CoA + H(+)

  Catalysed by CERS2cytosol

• a 1,3-diacyl-sn-glycerolReach from here →
  1. alpha 1,4-galactosyltransferase (P blood group) (LacCer pool)
  2. a globoside Gb3Cer (d18:1(4E)) + H2O = a lysoGb3(d18:1(4E)) + a fatty acid
  3. a 1,3-diacyl-sn-glycerol + H2O = a 1-acyl-sn-glycerol + a fatty acid + H(+)

  Catalysed by A4GALT, ASAH1, ABHD11cytosol

• a fatty acyl-CoAReach from here →
  1. alpha 1,4-galactosyltransferase (P blood group) (LacCer pool)
  2. a globoside Gb3Cer (d18:1(4E)) + H2O = a lysoGb3(d18:1(4E)) + a fatty acid
  3. a fatty acid + ATP + CoA = a fatty acyl-CoA + AMP + diphosphate

  Catalysed by A4GALT, ASAH1, ACSL3cytosol

• N-(9Z-octadecenoyl)-sphing-4-enineReach from here →
  1. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  2. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  3. N-(9Z-octadecenoyl)-sphing-4-enine + H2O = sphing-4-enine + (9Z)-octadecenoate

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 morecytosol

• a ganglioside GD3 (d18:1(4E))Reach from here →
  1. glucosylceramide pool to LacCer pool conversion
  2. ST3 beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)
  3. ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by ALG1L2, B4GALT5, B3GALT2, B4GALT3 and 33 moreendoplasmic reticulum, cytosolOne of 5 routes

• a ganglioside GT1a (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GM2)
  2. beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)
  3. ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by B3GALT4, ST3GAL1, ST3GAL4, ST3GAL2 and 1 morecytosolOne of 3 routes

• a ganglioside GQ1b (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GD2)
  2. beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)
  3. ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by B3GALT4, ST3GAL1, ST3GAL4, ST3GAL2 and 1 morecytosolOne of 3 routes

• a neolactoside IV(3)-beta-[Gal-beta-(1->3)-GlcNAc]-nLc4Cer(d18:1(4E))Reach from here →
  1. beta-1,4-galactosyltransferase (lc3Cer)
  2. a neolactoside nLc4Cer(d18:1(4E)) + UDP-N-acetyl-alpha-D-glucosamine = a neolactoside IV(3)-beta-GlcNAc-nLc4Cer(d18:1(4E)) + UDP + H(+)
  3. a neolactoside IV(3)-beta-GlcNAc-nLc4Cer(d18:1(4E)) + UDP-alpha-D-galactose = a neolactoside IV(3)-beta-[Gal-beta-(1->3)-GlcNAc]-nLc4Cer(d18:1(4E)) + UDP + H(+)

  Catalysed by B4GALT3, B4GALT4, B4GALT2, B4GALT1 and 2 morecytosolOne of 2 routes

• a ganglioside GM1b (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GM2)
  2. a ganglioside GM1 (d18:1(4E)) + H2O = a ganglioside GA1 (d18:1(4E)) + N-acetylneuraminate
  3. a ganglioside GA1 (d18:1(4E)) + CMP-N-acetyl-beta-neuraminate = a ganglioside GM1b (d18:1(4E)) + CMP + H(+)

  Catalysed by B3GALT4, NEU3, ST3GAL1, ST3GAL4 and 1 morecytosol

• a ganglioside GT1aalpha (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GM2)
  2. beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)
  3. a ganglioside GD1a (d18:1(4E)) + CMP-N-acetyl-beta-neuraminate = a ganglioside GT1aalpha (d18:1(4E)) + CMP + H(+)

  Catalysed by B3GALT4, ST3GAL1, ST3GAL4, ST3GAL2 and 1 morecytosol

• a ganglioside GQ1balpha (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GD2)
  2. beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)
  3. a ganglioside GT1b (d18:1(4E)) + CMP-N-acetyl-beta-neuraminate = a ganglioside GQ1balpha (d18:1(4E)) + CMP + H(+)

  Catalysed by B3GALT4, ST3GAL1, ST3GAL4, ST3GAL2 and 1 morecytosol

• a beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramideReach from here →
  1. UDP-glucose:alpha-D-galactose-1-phosphate uridylyltransferase
  2. alpha-D-galactose 1-phosphate + UDP-alpha-D-glucose = alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
  3. a beta-D-glucosylceramide + UDP-alpha-D-galactose = a beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide + UDP + H(+)

  Catalysed by GALT, B4GALT3, B4GALT1cytosol

• a ganglioside GM1Reach from here →
  1. UDP-glucose:alpha-D-galactose-1-phosphate uridylyltransferase
  2. alpha-D-galactose 1-phosphate + UDP-alpha-D-glucose = alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
  3. a ganglioside GM2 + UDP-alpha-D-galactose = a ganglioside GM1 + UDP + H(+)

  Catalysed by GALT, B3GALT4cytosol

• 1,2-dihexadecanoyl-sn-glycerolReach from here →
  1. UDP-alpha-D-galactose:N-acylsphingosine D-galactosyltransferase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. 1,2-dihexadecanoyl-sn-glycero-3-phosphocholine + an N-acylsphing-4-enine = 1,2-dihexadecanoyl-sn-glycerol + a sphingomyelin

  Catalysed by UGT8, GALC, SGMS2cytosol, lysosome

• 1-(9Z-octadecenoyl)-2-acyl-sn-3-glycerolReach from here →
  1. UDP-alpha-D-galactose:N-acylsphingosine D-galactosyltransferase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. 1-(9Z-octadecenoyl)-2-acyl-sn-3-glycerol + a sphingomyelin = a 1-(9Z-octadecenoyl)-2-acyl-sn-glycero-3-phosphocholine + an N-acylsphing-4-enine

  Catalysed by UGT8, GALC, SGMS1, SGMS2cytosol, lysosome

• N-(2-hydroxy-hexanoyl)-beta-D-galactosyl-sphing-4-enineReach from here →
  1. UDP-glucose:alpha-D-galactose-1-phosphate uridylyltransferase
  2. alpha-D-galactose 1-phosphate + UDP-alpha-D-glucose = alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
  3. N-(2-hydroxy-hexanoyl)-sphing-4-enine + UDP-alpha-D-galactose = N-(2-hydroxy-hexanoyl)-beta-D-galactosyl-sphing-4-enine + UDP + H(+)

  Catalysed by GALT, UGT8cytosol

• N-(2-hydroxyhexanoyl)-beta-D-galactosylsphinganineReach from here →
  1. UDP-glucose:alpha-D-galactose-1-phosphate uridylyltransferase
  2. alpha-D-galactose 1-phosphate + UDP-alpha-D-glucose = alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
  3. N-(2-hydroxy-hexanoyl)-sphinganine + UDP-alpha-D-galactose = N-(2-hydroxyhexanoyl)-beta-D-galactosylsphinganine + UDP + H(+)

  Catalysed by GALT, UGT8cytosol

• sphinga-4E,14Z-dienineReach from here →
  1. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  2. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase
  3. sphing-4-enine + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = sphinga-4E,14Z-dienine + 2 Fe(III)-[cytochrome b5] + 2 H2O

  Catalysed by FADS3cytosol

• a globoside Gb3CerReach from here →
  1. UDP-glucose:alpha-D-galactose-1-phosphate uridylyltransferase
  2. alpha-D-galactose 1-phosphate + UDP-alpha-D-glucose = alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
  3. a beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide + UDP-alpha-D-galactose = a globoside Gb3Cer + UDP + H(+)

  Catalysed by GALT, A4GALTcytosol

• a neolactoside nLc6CerReach from here →
  1. UDP-glucose:alpha-D-galactose-1-phosphate uridylyltransferase
  2. alpha-D-galactose 1-phosphate + UDP-alpha-D-glucose = alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
  3. a neolactoside IV(3)-beta-GlcNAc-nLc4Cer + UDP-alpha-D-galactose = a neolactoside nLc6Cer + UDP + H(+)

  Catalysed by GALT, B4GALT3, B4GALT1cytosol