CHEBI:12947

Three steps

• (S)-malateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. transport of malate and sulfate (cytosol to mitochondria)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 1 morelysosome, mitochondrial matrix, cytosolOne of 4 routes

• hexadecanoic acidReach 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

• malonateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. malonate(in) + sulfate(out) = malonate(out) + sulfate(in)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 3 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• 1-acylglycerone 3-phosphateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:glycerone-phosphate O-acyltransferase

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

• keto-D-fructose 6-phosphateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. ATP:D-glucose 6-phosphotransferase
  3. D-glucose-6-phosphate aldose-ketose-isomerase

  Catalysed by GALC, HK1, GCK, HK2 and 3 morelysosome, cytosol

• thioredoxin dithiolReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. dATP:D-glucose 6-phosphotransferase
  3. 2-Deoxyuridine 5-Diphosphate:Oxidized-Thioredoxin 2-Oxidoreductase

  Catalysed by GALC, HK1, HK2, HK3 and 6 morelysosome, cytosol, mitochondrial matrixOne of 2 routes

• phosphatidylethanolamineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-acyl-sn-glycero-3-phosphoethanolamine O-acyltransferase

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 6 morelysosome, Golgi apparatus, cytosolOne of 2 routes

• CHEBI:16039Reach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. ITP:D-glucose 6-phosphotransferase
  3. ATP:IDP phosphotransferase

  Catalysed by GALC, HK1, HK2, HK3 and 6 morelysosome, cytosolOne of 3 routes

• 1,2-diacyl-sn-glycero-3-phosphocholine(1+)Reach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-acyl-sn-glycero-3-phosphocholine O-acyltransferase

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

• CHEBI:16284Reach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. dATP:D-glucose 6-phosphotransferase
  3. ATP:dADP phosphotransferase

  Catalysed by GALC, HK1, HK2, HK3 and 6 morelysosome, cytosolOne of 16 routes

• phosphatidic acidReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:1-acyl-sn-glycerol-3-phosphate 2-O-acyltransferase

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 10 morelysosome, Golgi apparatus, mitochondrial matrixOne of 9 routes

• CHEBI:16436Reach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. ceramide cholinephosphotransferase (CDP-choline)

  Catalysed by GALC, SGMS1, SGMS2lysosome, Golgi apparatus, endoplasmic reticulum

• alpha,alpha-trehaloseReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. D-glucose 1-epimerase
  3. alpha,alpha-trehalose + H2O = alpha-D-glucose + beta-D-glucose

  Catalysed by GALC, GALM, TREHlysosome, cytosol

• pregnenoloneReach 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. 6 reduced [adrenodoxin] + cholesterol + 3 O2 + 6 H(+) = 4-methylpentanal + pregnenolone + 6 oxidized [adrenodoxin] + 4 H2O

  Catalysed by GALC, GBA1, GBA2, CYP11A1lysosome

• quinolinic acidReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. Transport of Quinolinic Acid

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

• psychosineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. UDP-alpha-D-galactose:sphingosine 1-beta-galactosyltransferase

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

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

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

• oxalic acidReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. transport of oxalate and sulfate (cytosol to extracellular)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 1 morelysosome, extracellular space, cytosol

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

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

• a beta-D-GlcNAc-(1->3)-beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))Reach from here →
  1. D-galactosyl-N-acylsphingosine galactohydrolase
  2. beta-galactosidase (galactose)
  3. UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase (LacCer pool)

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

• CHEBI:17202Reach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. ITP:D-glucose 6-phosphotransferase
  3. IDP phosphohydrolase

  Catalysed by GALC, HK1, HK2, HK3 and 9 morelysosome, cytosol, extracellular space

• linoleic acidReach 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

• sulfiteReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. sulfite(in) + sulfate(out) = sulfite(out) + sulfate(in)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 2 morelysosome, inner side of the membrane, outer side of the membraneOne of 2 routes

• a 2-acylglycerolReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. a 1,2-diacyl-sn-glycerol + H2O = a 2-acylglycerol + a fatty acid + H(+)

  Catalysed by GALC, SGMS1, SGMS2, DAGLB and 2 morelysosome, Golgi apparatus

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

  Catalysed by GALC, SGMS1, SGMS2, LIPF and 14 morelysosome, Golgi apparatus, extracellular spaceOne of 9 routes

• 1-(alk-1-enyl)-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-alkenylglycerophosphoethanolamine O-acyltransferase

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

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

  Catalysed by GALC, UGCG, GBA1, GBA2lysosomeOne of 8 routes

• 7alpha-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. cholesterol + reduced [NADPH--hemoprotein reductase] + O2 = 7alpha-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by GALC, GBA1, GBA2, CYP7A1lysosome

• 3,3'-diiodo-L-thyronine sulfateReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. Steryl-Sulfatase

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 1 morelysosome, Golgi apparatusOne of 2 routes

• 26-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. cholesterol + reduced [NADPH--hemoprotein reductase] + O2 = 26-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by GALC, GBA1, GBA2, CYP3A4lysosome

• a triacylglycerolReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase

  Catalysed by GALC, ASAH1, ACER2, ACER1 and 6 morelysosome, Golgi apparatus, cytosolOne of 2 routes

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

  Catalysed by GALC, GALMlysosome, cytosol

• 4-methylpentanalReach 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. 6 reduced [adrenodoxin] + cholesterol + 3 O2 + 6 H(+) = 4-methylpentanal + pregnenolone + 6 oxidized [adrenodoxin] + 4 H2O

  Catalysed by GALC, GBA1, GBA2, CYP11A1lysosome

• fumaric acidReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. transport of fumarate and sulfate (cytosol to mitochondria)

  Catalysed by GAL3ST1, ARSA, ARSD, ARSL and 1 morelysosome, mitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by GALC, HK1, HK2, HK3 and 7 morelysosome, cytosolOne of 3 routes

• CHEBI:18132Reach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. Sphingomyelin cholinephosphohydrolase

  Catalysed by GALC, SGMS1, SGMS2, SMPD2 and 4 morelysosome, Golgi apparatus, cytosolOne of 4 routes

• dodecanoateReach 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

• a globoside Gb3Cer (d18:1(4E))Reach from here →
  1. D-galactosyl-N-acylsphingosine galactohydrolase
  2. beta-galactosidase (galactose)
  3. alpha 1,4-galactosyltransferase (P blood group) (LacCer pool)

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

• D-gluconateReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. D-glucose + NAD(+) = D-glucono-1,5-lactone + NADH + H(+)
  3. D-glucono-1,5-lactone + H2O = D-gluconate + H(+)

  Catalysed by GALC, H6PD, RGNlysosome

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

  Catalysed by GAL3ST1, ARSA, GBA1, GBA2lysosomeOne of 3 routes

• cholesteryl 3-beta-D-xylosideReach 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 beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine

  Catalysed by GALC, GBA1, GBA2, GBA3lysosomeOne of 3 routes

• (11Z)-docosenoyl-CoA(4-)Reach 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

• (7Z)-tetradecenoyl-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

• NAADPHReach from here →
  1. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  2. D-glucose + ADP = D-glucose 6-phosphate + AMP + H(+)
  3. NAADP(+) + D-glucose 6-phosphate = NAADPH + 6-phospho-D-glucono-1,5-lactone + H(+)

  Catalysed by GALC, ADPGK, G6PDlysosome

• docosanoateReach 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

• octadecanoateReach 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-linolenic acidReach 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

• folic acidReach from here →
  1. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  2. Sulfatide sulfohydrolase
  3. Folate Transport by Oats

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

• a ganglioside GA2 (d18:1(4E))Reach from here →
  1. D-galactosyl-N-acylsphingosine galactohydrolase
  2. beta-galactosidase (galactose)
  3. beta-1,4-N-acetyl-galactosaminyltransferase (LacCer pool)

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

• elaidic acidReach 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, cytosolOne of 13 routes