an N-acyl-1-beta-D-(3-O-sulfo)-galactosyl-sphing-4-enine

Three steps

• L-tyrosineReach from here →
  1. Sulfatide sulfohydrolase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)

  Catalysed by ARSA, ARSD, ARSL, SLC25A14 and 2 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(omega),N(omega)-dimethyl-L-arginineReach from here →
  1. Sulfatide sulfohydrolase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate

  Catalysed by ARSA, ARSD, ARSL, SLC25A14 and 2 morelysosome, inner side of the membrane, outer side of the membrane

• alpha-D-galactose 1-phosphateReach from here →
  1. Sulfatide sulfohydrolase
  2. D-galactosyl-N-acylsphingosine galactohydrolase
  3. galactokinase (galactose)

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

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

  Catalysed by ARSA, ARSD, ARSL, GALC and 4 morelysosome, Golgi apparatusOne of 2 routes

• (2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinateReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole AMP-lyase

  Catalysed by ARSA, ARSD, ARSL, SLC25A10 and 1 morelysosome, mitochondrial matrix, cytosolOne of 4 routes

• kynurenateReach from here →
  1. Sulfatide sulfohydrolase
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)

  Catalysed by ARSA, ARSD, ARSL, SLC25A35 and 2 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by ARSA, ARSD, ARSL, SGSHlysosome

• S-sulfanylglutathioneReach from here →
  1. Sulfatide sulfohydrolase
  2. sulfite(in) + sulfate(out) = sulfite(out) + sulfate(in)
  3. S-sulfanylglutathione + O2 + H2O = sulfite + glutathione + 2 H(+)

  Catalysed by ARSA, ARSD, ARSL, SLC25A14 and 2 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. Sulfatide sulfohydrolase
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)

  Catalysed by ARSA, ARSD, ARSL, SLC25A35 and 3 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• 4-aminobutanoateReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 1 morelysosome, extracellular space, cytosolOne of 4 routes

• prostaglandin E2Reach from here →
  1. Sulfatide sulfohydrolase
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. prostaglandin E2(out) + a dicarboxylate(in) = prostaglandin E2(in) + a dicarboxylate(out)

  Catalysed by ARSA, ARSD, ARSL, SLC25A35 and 2 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• N-benzoylglycineReach from here →
  1. Sulfatide sulfohydrolase
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. N-benzoylglycine(out) + a dicarboxylate(in) = N-benzoylglycine(in) + a dicarboxylate(out)

  Catalysed by ARSA, ARSD, ARSL, SLC25A35 and 3 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• 3-sulfino-L-alanineReach from here →
  1. Sulfatide sulfohydrolase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)

  Catalysed by ARSA, ARSD, ARSL, SLC25A14 and 3 morelysosome, inner side of the membrane, outer side of the membrane, mitochondrial matrix, cytosolOne of 4 routes

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

  Catalysed by ARSA, ARSD, ARSL, GALC and 1 morelysosomeOne of 2 routes

• ubiquinol-10Reach from here →
  1. Sulfatide sulfohydrolase
  2. sulfite(in) + sulfate(out) = sulfite(out) + sulfate(in)
  3. ubiquinone-10 + hydrogen sulfide + sulfite + 2 H(+) = ubiquinol-10 + thiosulfate

  Catalysed by ARSA, ARSD, ARSL, SLC25A14 and 2 morelysosome, inner side of the membrane, outer side of the membrane

• an N-acylsphing-4-enine 1-phosphoethanolamineReach from here →
  1. Sulfatide sulfohydrolase
  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 ARSA, ARSD, ARSL, GALC and 3 morelysosome

• 1-(beta-D-galactosyl)-N-dodecanoylsphing-4-enineReach from here →
  1. 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(+)
  2. a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-galactoside + an N-acylsphing-4-enine
  3. 1-(beta-D-galactosyl)-N-dodecanoylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-galactoside + N-dodecanoylsphing-4-enine

  Catalysed by ARSA, GBA1, GBA2

• 1,2-dihexadecanoyl-sn-glycerolReach from here →
  1. Sulfatide sulfohydrolase
  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 ARSA, ARSD, ARSL, GALC and 1 morelysosome

• 1-(9Z-octadecenoyl)-2-acyl-sn-3-glycerolReach from here →
  1. Sulfatide sulfohydrolase
  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 ARSA, ARSD, ARSL, GALC and 2 morelysosome

• 16alpha-hydroxydehydroepiandrosterone 3-sulfateReach from here →
  1. Sulfatide sulfohydrolase
  2. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)
  3. dehydroepiandrosterone 3-sulfate + reduced [NADPH--hemoprotein reductase] + O2 = 16alpha-hydroxydehydroepiandrosterone 3-sulfate + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

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

• an N-acetyl-alpha-neuraminosyl-(2->8)-N-acetyl-alpha-neuraminosyl-(2->3)-beta-D-galactosyl-(1<->1')-N-acylsphing-4-enineReach from here →
  1. 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(+)
  2. a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + CMP-N-acetyl-beta-neuraminate = a ganglioside GM4 (d18:1(4E)) + CMP + H(+)
  3. a ganglioside GM4 (d18:1(4E)) + CMP-N-acetyl-beta-neuraminate = an N-acetyl-alpha-neuraminosyl-(2->8)-N-acetyl-alpha-neuraminosyl-(2->3)-beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + CMP + H(+)

  Catalysed by ARSA, ST3GAL5, ST8SIA6