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

Three steps

• L-sepiapterinReach from here →
  1. Sulfatide sulfohydrolase
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. L-sepiapterin(out) + a dicarboxylate(in) = L-sepiapterin(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

• a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enineReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. an N-acylsphing-4-enine + UDP-alpha-D-glucose = a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + UDP + H(+)

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

• CHEBI:240943Reach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. 2-Methylbutyrylglycine Transport, Extracellular

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

• a quinolReach 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

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

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

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

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

• beta-D-galactoseReach from here →
  1. Sulfatide sulfohydrolase
  2. D-galactosyl-N-acylsphingosine galactohydrolase
  3. galactose[n] = beta-D-galactose[n]

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

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

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

• a fatty acidReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. an N-acylsphing-4-enine + H2O = sphing-4-enine + a fatty acid

  Catalysed by ARSA, ARSD, ARSL, GALC and 6 morelysosome

• K(+)Reach from here →
  1. Sulfatide sulfohydrolase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. K(+)(in) + L-aspartate(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-aspartate(in) + 3 Na(+)(in) + H(+)(in)

  Catalysed by ARSA, ARSD, ARSL, SLC25A14 and 6 morelysosome, inner side of the membrane, outer side of the membraneOne of 4 routes

• Asp-tRNA(Asp)Reach from here →
  1. Sulfatide sulfohydrolase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. L-Aspartate:tRNA(Asp) ligase (AMP-forming)

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

• hydrogen sulfideReach from here →
  1. Sulfatide sulfohydrolase
  2. thiosulfate(in) + sulfate(out) = thiosulfate(out) + sulfate(in)
  3. 3alpha-hydroxysteroid 3-dehydrogenase (Si-specific) (H2S2O3)

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

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

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

• citric acidReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. Citrate Transport, Mitochondrial

  Catalysed by ARSA, ARSD, ARSL, SLC25A10 and 1 morelysosome, mitochondrial matrix, cytosolOne of 10 routes

• (indol-3-yl)acetateReach from here →
  1. Sulfatide sulfohydrolase
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. (indol-3-yl)acetate(out) + a dicarboxylate(in) = (indol-3-yl)acetate(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

• isocitric acidReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by ARSA, ARSD, ARSL, SLC25A10 and 1 morelysosome, mitochondrial matrix, cytosolOne of 6 routes

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

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

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

• cis-aconitic acidReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by ARSA, ARSD, ARSL, SLC25A10 and 1 morelysosome, mitochondrial matrix, cytosolOne of 6 routes

• N-carbamoyl-L-aspartateReach from here →
  1. Sulfatide sulfohydrolase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. carbamoyl phosphate + L-aspartate = N-carbamoyl-L-aspartate + phosphate + H(+)

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

• a phenolReach from here →
  1. Sulfatide sulfohydrolase
  2. an aryl sulfate + H2O = a phenol + sulfate + H(+)
  3. an aryl sulfate + H2O = a phenol + sulfate + H(+)

  Catalysed by ARSA, ARSD, ARSL, ARSF and 4 morelysosome

• fatty acidReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. N-Acylsphingosine amidohydrolase

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

• alpha-lactoseReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. UDP-alpha-D-galactose:D-glucose 4-beta-D-galactosyltransferase

  Catalysed by ARSA, ARSD, ARSL, GALC and 3 morelysosome, Golgi apparatus

• glyoxylateReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)

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

• D-glucopyranose 6-phosphateReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. ATP:D-glucose 6-phosphotransferase

  Catalysed by ARSA, ARSD, ARSL, GALC and 5 morelysosome, cytosolOne of 5 routes

• L-erythro-7,8-dihydrobiopterinReach from here →
  1. Sulfatide sulfohydrolase
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. L-erythro-7,8-dihydrobiopterin(out) + a dicarboxylate(in) = L-erythro-7,8-dihydrobiopterin(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

• N-acetyl-beta-neuraminic acidReach 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. Acylneuraminyl hydrolase

  Catalysed by ARSA, ST3GAL5, NEU4, NEU1 and 2 morelysosome

• (5-hydroxyindol-3-yl)acetaldehydeReach from here →
  1. Sulfatide sulfohydrolase
  2. Transport of 5-Hydroxyindoleacetate
  3. (5-hydroxyindol-3-yl)acetaldehyde + NAD(+) + H2O = (5-hydroxyindol-3-yl)acetate + NADH + 2 H(+)

  Catalysed by ARSA, ARSD, ARSL, SLC4A1 and 1 morelysosome, extracellular space, cytosol

• taurineReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3

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

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

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

• (S)-malyl-CoAReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of malate and sulfate (cytosol to mitochondria)
  3. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate

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

• prostaglandin F2alphaReach from here →
  1. Sulfatide sulfohydrolase
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. prostaglandin F2alpha(out) + a dicarboxylate(in) = prostaglandin F2alpha(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

• 7,8-dihydrofolateReach from here →
  1. Sulfatide sulfohydrolase
  2. Folate Transport by Oats
  3. dihydrofolate:NAD+ oxidoreductase

  Catalysed by ARSA, ARSD, ARSL, SLC22A8 and 3 morelysosome, extracellular space, cytosolOne of 3 routes

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

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

• N(6)-(1,2-dicarboxyethyl)-AMPReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of fumarate and sulfate (cytosol to mitochondria)
  3. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)

  Catalysed by ARSA, ARSD, ARSL, SLC25A10 and 2 morelysosome, mitochondrial matrix, cytosolOne of 4 routes

• an N-acylsphing-4-enine 1-phosphateReach from here →
  1. Sulfatide sulfohydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. an N-acylsphing-4-enine + ATP = an N-acylsphing-4-enine 1-phosphate + ADP + H(+)

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

• guanidinoacetateReach from here →
  1. Sulfatide sulfohydrolase
  2. transport of chloride and sulfate (cytosol to extracellular)
  3. Transport of Guanidinoacetic Acid

  Catalysed by ARSA, ARSD, ARSL, SLC26A6lysosome, extracellular space, cytosol

• 3',5'-cyclic GMPReach from here →
  1. Sulfatide sulfohydrolase
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. 3',5'-cyclic GMP(out) + a dicarboxylate(in) = 3',5'-cyclic GMP(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

• sphing-4-enineReach 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 4 routes

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

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

• adenosine 5'-phosphoramidateReach from here →
  1. Sulfatide sulfohydrolase
  2. ATP:sulfate adenylyltransferase
  3. adenosine 5'-phosphosulfate + NH4(+) = adenosine 5'-phosphoramidate + sulfate + 2 H(+)

  Catalysed by ARSA, ARSD, ARSL, PAPSS1 and 2 morelysosome, cytosol

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

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

• glutathioneReach from here →
  1. Sulfatide sulfohydrolase
  2. sulfite(in) + sulfate(out) = sulfite(out) + sulfate(in)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

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

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

  Catalysed by ARSA, ARSD, ARSL, SLC26A6 and 2 morelysosome, extracellular space, cytosolOne of 4 routes

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

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

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

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

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach from here →
  1. Sulfatide sulfohydrolase
  2. L-aspartate(in) + sulfate(out) = L-aspartate(out) + sulfate(in)
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

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

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

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

• 3',5'-cyclic AMPReach from here →
  1. Sulfatide sulfohydrolase
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. 3',5'-cyclic AMP(out) + a dicarboxylate(in) = 3',5'-cyclic AMP(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

• glutathione disulfideReach from here →
  1. Sulfatide sulfohydrolase
  2. thiosulfate(in) + sulfate(out) = thiosulfate(out) + sulfate(in)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by ARSA, ARSD, ARSL, SLC25A14 and 4 morelysosome, inner side of the membrane, outer side of the membrane