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

Three steps

• nicotinic acid D-ribonucleotideReach from here →
  1. Sulfatide sulfohydrolase
  2. Transport of Quinolinic Acid
  3. Nicotinate-nucleotide:pyrophosphate phosphoribosyltransferase (carboxylating)

  Catalysed by ARSA, ARSD, ARSL, SLC22A6 and 1 morelysosome, extracellular space, cytosol

• N-carbamoyl-L-aspartic acidReach from here →
  1. Sulfatide sulfohydrolase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. carbamoyl-phosphate:L-aspartate carbamoyltransferase

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

• beta-D-glucoseReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. D-glucose 1-epimerase

  Catalysed by ARSA, ARSD, ARSL, GALC and 1 morelysosome, cytosol

• D-galactono-1,5-lactoneReach 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 + H2O = D-galactose + an N-acylsphing-4-enine
  3. D-galactose + NADP(+) = D-galactono-1,5-lactone + NADPH + H(+)

  Catalysed by ARSA, GBA1, GALC, GBA3 and 2 more

• CHEBI:15971Reach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of chloride and histidine (cytosol to extracellular)

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 1 morelysosome, extracellular space, cytosol

• androsteroneReach from here →
  1. Sulfatide sulfohydrolase
  2. steroid sulfatase (androsterone sulfate)
  3. steroid sulfatase (androsterone sulfate)

  Catalysed by ARSA, ARSD, ARSL, STSlysosome, cytosolOne of 3 routes

• cholesterolReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine

  Catalysed by ARSA, ARSD, ARSL, GALC and 2 morelysosomeOne of 5 routes

• CHEBI:16174Reach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. dATP:D-glucose 6-phosphotransferase

  Catalysed by ARSA, ARSD, ARSL, GALC and 4 morelysosome, cytosol

• D-glucono-1,5-lactoneReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. D-glucose + NAD(+) = D-glucono-1,5-lactone + NADH + H(+)

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

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

• 17beta-hydroxy-5alpha-androstan-3-oneReach from here →
  1. Sulfatide sulfohydrolase
  2. steroid sulfatase (5-alpha-dihydrotestosterone)
  3. steroid sulfatase (5-alpha-dihydrotestosterone)

  Catalysed by ARSA, ARSD, ARSL, STSlysosome, cytosolOne of 3 routes

• iodideReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of chloride and iodide (cytosol to extracellular)

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 1 morelysosome, extracellular space, cytosol

• ubiquinonesReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. succinate:quinone oxidoreductase

  Catalysed by ARSA, ARSD, ARSL, SLC25A10 and 4 morelysosome, mitochondrial matrix, cytosol

• L-valineReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of chloride and valine (cytosol to extracellular)

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

• oxaloacetateReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. transport of malate and OAA (cytosol to mitochondria)

  Catalysed by ARSA, ARSD, ARSL, SLC25A10lysosome, mitochondrial matrix, cytosolOne of 11 routes

• L-arginineReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of arginine and chloride (cytosol to extracellular)

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 1 morelysosome, extracellular space, cytosolOne of 2 routes

• protein polypeptide chainReach from here →
  1. Sulfatide sulfohydrolase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. PROTEIN_BS

  Catalysed by ARSA, ARSD, ARSL, SLC25A14 and 1 morelysosome, inner side of the membrane, outer side of the membrane, cytosol

• pregnenoloneReach from here →
  1. Sulfatide sulfohydrolase
  2. Steryl-Sulfatase
  3. Pregnenolone-sulfate sulfohydrolase

  Catalysed by ARSA, ARSD, ARSL, STSlysosome, Golgi apparatus, cytosolOne of 4 routes

• galactitolReach from here →
  1. Sulfatide sulfohydrolase
  2. D-galactosyl-N-acylsphingosine galactohydrolase
  3. galactitol:NADP+ 1-oxidoreductase

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

• 3-fumarylpyruvateReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by ARSA, ARSD, ARSL, SLC25A10 and 1 morelysosome, mitochondrial matrix, cytosol

• CHEBI:16857Reach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Transport of L-Threonine by Atb0 Transporter

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 1 morelysosome, extracellular space, cytosol

• ferrocytochrome cReach from here →
  1. Sulfatide sulfohydrolase
  2. sulfite(in) + sulfate(out) = sulfite(out) + sulfate(in)
  3. sulfite:ferricytochrome-c oxidoreductase

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

• N-acetyl-L-aspartateReach from here →
  1. Sulfatide sulfohydrolase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. acetyl-CoA:L-aspartate N-acetyltransferase

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

• L-isoleucineReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of chloride and isoleucine (cytosol to extracellular)

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 1 morelysosome, extracellular space, cytosolOne of 2 routes

• CHEBI:17203Reach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Transport of L-Proline by the Apical Imino Amino Acid Transporters

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 2 morelysosome, extracellular space, cytosolOne of 3 routes

• estroneReach from here →
  1. Sulfatide sulfohydrolase
  2. estrone 3-sulfate + H2O = estrone + sulfate + H(+)
  3. Estrone 3-sulfate sulfohydrolase

  Catalysed by ARSA, ARSD, ARSL, STSlysosome, cytosolOne of 3 routes

• propanoateReach from here →
  1. Sulfatide sulfohydrolase
  2. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)
  3. propanoate(out) + dehydroepiandrosterone 3-sulfate(in) = propanoate(in) + dehydroepiandrosterone 3-sulfate(out)

  Catalysed by ARSA, ARSD, ARSL, STS and 1 morelysosome, inner side of the membrane, outer side of the membraneOne of 4 routes

• enol-oxaloacetateReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by ARSA, ARSD, ARSL, SLC25A10 and 2 morelysosome, mitochondrial matrix, cytosol

• L-cysteineReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of chloride and cysteine (cytosol to extracellular)

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 1 morelysosome, extracellular space, cytosolOne of 2 routes

• a sphingomyelinReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. ceramide:phosphatidylcholine cholinephosphotransferase

  Catalysed by ARSA, ARSD, ARSL, GALC and 2 morelysosome, Golgi apparatusOne of 8 routes

• 3,3'-diiodo-L-thyronineReach from here →
  1. Sulfatide sulfohydrolase
  2. Steryl-Sulfatase
  3. Steryl-Sulfatase

  Catalysed by ARSA, ARSD, ARSL, STSlysosome, Golgi apparatusOne of 3 routes

• lactoseReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. D-glucose + UDP-alpha-D-galactose = lactose + UDP + H(+)

  Catalysed by ARSA, ARSD, ARSL, GALC and 2 morelysosome

• glycine betaineReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of betaine and chloride (cytosol to extracellular)

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 1 morelysosome, extracellular space, cytosolOne of 3 routes

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

• CHEBI:17808Reach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. ITP:D-glucose 6-phosphotransferase

  Catalysed by ARSA, ARSD, ARSL, GALC and 4 morelysosome, cytosol

• a 1,2-diacyl-sn-glycerolReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. ceramide:phosphatidylcholine cholinephosphotransferase

  Catalysed by ARSA, ARSD, ARSL, GALC and 2 morelysosome, Golgi apparatusOne of 6 routes

• CHEBI:17895Reach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Transport of L-Tyrosine by Atb0 Transporter

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 1 morelysosome, extracellular space, cytosolOne of 2 routes

• D-glucitolReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. Aldehyde Reductase

  Catalysed by ARSA, ARSD, ARSL, GALClysosome, cytosolOne of 3 routes

• a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))Reach from here →
  1. Sulfatide sulfohydrolase
  2. D-galactosyl-N-acylsphingosine galactohydrolase
  3. beta-galactosidase (galactose)

  Catalysed by ARSA, ARSD, ARSL, GALC and 3 morelysosome, extracellular space

• butanoateReach from here →
  1. Sulfatide sulfohydrolase
  2. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)
  3. dehydroepiandrosterone 3-sulfate(in) + butanoate(out) = dehydroepiandrosterone 3-sulfate(out) + butanoate(in)

  Catalysed by ARSA, ARSD, ARSL, STS and 1 morelysosome, inner side of the membrane, outer side of the membraneOne of 4 routes

• CHEBI:18019Reach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of chloride and lysine (cytosol to extracellular)

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 1 morelysosome, extracellular space, cytosol

• thiocyanateReach from here →
  1. Sulfatide sulfohydrolase
  2. thiosulfate(in) + sulfate(out) = thiosulfate(out) + sulfate(in)
  3. thiosulfate:cyanide sulfurtranserase

  Catalysed by ARSA, ARSD, ARSL, SLC25A14 and 5 morelysosome, inner side of the membrane, outer side of the membrane, mitochondrial matrixOne of 2 routes

• 4-fumarylacetoacetateReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)

  Catalysed by ARSA, ARSD, ARSL, SLC25A10 and 1 morelysosome, mitochondrial matrix, cytosol

• L-glutamineReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Transport of L-Glutamine by Atb0 Transporter

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 1 morelysosome, extracellular space, cytosolOne of 3 routes

• CHEBI:18095Reach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. transport of trans-4-hydroxy-L-proline (cytosol to extracellular)

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 2 morelysosome, extracellular space, cytosol

• N-carbamoyl-beta-alanineReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

  Catalysed by ARSA, ARSD, ARSL, SLC26A6lysosome, extracellular space, cytosolOne of 2 routes

• D-glucosyl-N-acylsphingosineReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. UDP-glucose:N-acylsphingosine D-glucosyltransferase

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

• hydrogen cyanideReach from here →
  1. Sulfatide sulfohydrolase
  2. sulfite(in) + sulfate(out) = sulfite(out) + sulfate(in)
  3. thiosulfate:cyanide sulfurtranserase

  Catalysed by ARSA, ARSD, ARSL, SLC25A14 and 3 morelysosome, inner side of the membrane, outer side of the membrane, mitochondrial matrixOne of 2 routes

• a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enineReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine

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

• 3-carboxy-4-methyl-5-propyl-2-furanpropanoateReach from here →
  1. Sulfatide sulfohydrolase
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. 3-carboxy-4-methyl-5-propyl-2-furanpropanoate(out) + a dicarboxylate(in) = 3-carboxy-4-methyl-5-propyl-2-furanpropanoate(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