a ganglioside GM4 (d18:1(4E))

Three steps

• dTDPReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. pyruvate kinase (dTDP)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 3 morelysosome, cytosol

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

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosolOne of 2 routes

• a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enineReach from here →
  1. Acylneuraminyl hydrolase
  2. 3'-Phosphoadenylylsulfate:galactosylceramide 3'-sulfotransferase
  3. 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(+)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosomeOne of 3 routes

• a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enineReach from here →
  1. Acylneuraminyl hydrolase
  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 NEU4, NEU1, NEU3, NEU2 and 3 morelysosomeOne of 2 routes

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

• 5-(3,3'-dimethylguanidino)-2-oxopentanoateReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

• 5-(3-methylguanidino)-2-oxopentanoateReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. N(omega)-methyl-L-arginine + pyruvate = 5-(3-methylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

• an alpha-Neu5Ac-(2->8)-alpha-Neu5Ac-(2->3)-beta-D-Gal-(1->4)-beta-D-GlcNAc derivativeReach from here →
  1. Acylneuraminyl hydrolase
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. an N-acetyl-alpha-neuraminyl-(2->3)-beta-D-galactosyl-(1->4)-N-acetyl-beta-D-glucosaminyl derivative + CMP-N-acetyl-beta-neuraminate = an alpha-Neu5Ac-(2->8)-alpha-Neu5Ac-(2->3)-beta-D-Gal-(1->4)-beta-D-GlcNAc derivative + CMP + H(+)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, nucleus

• alpha-Neu5Ac-(2->8)-alpha-Neu5Ac-(2->3)-beta-D-Gal-(1->4)-6S-D-GlcNAcReach from here →
  1. Acylneuraminyl hydrolase
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. alpha-Neu5Ac-(2->3)-beta-D-Gal-(1->4)-6S-D-GlcNAc + CMP-N-acetyl-beta-neuraminate = alpha-Neu5Ac-(2->8)-alpha-Neu5Ac-(2->3)-beta-D-Gal-(1->4)-6S-D-GlcNAc + CMP + H(+)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, nucleus

• a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enineReach from here →
  1. Acylneuraminyl hydrolase
  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 NEU4, NEU1, NEU3, NEU2 and 2 morelysosomeOne of 2 routes

• CMP-N-acetyl-9-O-acetyl-beta-neuraminateReach from here →
  1. Acylneuraminyl hydrolase
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. CMP-N-acetyl-beta-neuraminate + acetyl-CoA = CMP-N-acetyl-9-O-acetyl-beta-neuraminate + CoA

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, nucleus

• CMP-N-acetyl-7-O-acetyl-beta-neuraminateReach from here →
  1. Acylneuraminyl hydrolase
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. CMP-N-acetyl-beta-neuraminate + acetyl-CoA = CMP-N-acetyl-7-O-acetyl-beta-neuraminate + CoA

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, nucleus

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

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, nucleusOne of 2 routes

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

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 7 morelysosome

• acetateReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. transport of acetate and pyruvate (cytosol to extracellular)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 6 morelysosome, cytosol, extracellular spaceOne of 2 routes

• formic acidReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. transport of formate and pyruvate (cytosol to extracellular)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 6 morelysosome, cytosol, extracellular spaceOne of 2 routes

• propionic acidReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. transport of propanoate and pyruvate (cytosol to extracellular)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 7 morelysosome, cytosol, extracellular spaceOne of 2 routes

• butyric acidReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. transport of butyrate and pyruvate (cytosol to extracellular)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 7 morelysosome, cytosol, extracellular spaceOne of 2 routes

• 3-hydroxypyruvic acidReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. L-Serine:pyruvate aminotransferase

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosolOne of 2 routes

• 3-oxopropanoateReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

• D-serineReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. D-serine = pyruvate + NH4(+)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

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

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome

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

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 4 morelysosome, Golgi apparatus

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

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 6 morelysosome, cytosolOne of 5 routes

• (R)-lactic acidReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (R)-Lactate:NAD+ oxidoreductase

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

• (S)-lactic acidReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (S)-Lactate:NAD+ oxidoreductase

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 3 morelysosome, cytosol, mitochondrial matrixOne of 5 routes

• 2-iminopropanoateReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

• phosphoenolpyruvic acidReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. pyruvate kinase (dTDP)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 3 morelysosome, cytosol

• N-acetyl-D-glucosamineReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. N-acetyl-D-glucosamine 2-epimerase

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

• a 3-acylpyruvateReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

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

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 3 morelysosome

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

• 2-methyl-3-oxopropanoateReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

• sphing-4-enineReach from here →
  1. Acylneuraminyl hydrolase
  2. galactosylceramidase (D-galactosyl-N-acylsphingosine)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 5 morelysosome, Golgi apparatusOne of 4 routes

• L-methionineReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 3 morelysosome, cytosol

• L-alanineReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol, mitochondrial matrixOne of 18 routes

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

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 3 morelysosome, cytosolOne of 2 routes

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

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 5 morelysosome, Golgi apparatusOne of 2 routes

• aldehydo-N-acetyl-D-mannosamine 6-phosphateReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. ATP:N-acyl-D-mannosamine 6-phosphotransferase

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosolOne of 2 routes

• a neolactoside IV(3)-alpha-NeuAc-nLc4Cer(d18:1(4E))Reach from here →
  1. Acylneuraminyl hydrolase
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. ST3 beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, nucleus, cytosolOne of 3 routes

• (3S)-citramalyl-CoAReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

• 5-amino-2-oxopentanoateReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

• a ganglioside GM3 (d18:1(4E))Reach from here →
  1. Acylneuraminyl hydrolase
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. ST3 beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, nucleus, cytosolOne of 3 routes

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

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosomeOne of 2 routes

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

• a ganglioside GM2 (d18:1(4E))Reach from here →
  1. Acylneuraminyl hydrolase
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. lactosylceramide alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 3 morelysosome, nucleus, cytosol

• (4S)-4-hydroxy-2-oxoglutarateReach from here →
  1. Acylneuraminyl hydrolase
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (4S)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, cytosol

• ganglioside GM1 (d18:1(4E)/18:0)Reach from here →
  1. Acylneuraminyl hydrolase
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. ganglioside GA1 (d18:1(4E)/18:0) + CMP-N-acetyl-beta-neuraminate = ganglioside GM1 (d18:1(4E)/18:0) + CMP + H(+)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, nucleus

• an N-acylsphing-4-enine 1-phosphoethanolamineReach from here →
  1. Acylneuraminyl hydrolase
  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 NEU4, NEU1, NEU3, NEU2 and 4 morelysosome

• a ganglioside GM1 (d18:1(4E))Reach from here →
  1. Acylneuraminyl hydrolase
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. ST3 beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morelysosome, nucleus, cytosol