a ganglioside GM2 (d18:1(4E))

Three steps

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol

• 5-(3,3'-dimethylguanidino)-2-oxopentanoateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol

• 5-(3-methylguanidino)-2-oxopentanoateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol

• an alpha-Neu5Ac-(2->8)-alpha-Neu5Ac-(2->3)-beta-D-Gal-(1->4)-beta-D-GlcNAc derivativeReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol, nucleus

• alpha-Neu5Ac-(2->8)-alpha-Neu5Ac-(2->3)-beta-D-Gal-(1->4)-6S-D-GlcNAcReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol, nucleus

• a ganglioside Ac-O-7-GD3(d18:1(4E))Reach from here →
  1. beta-N-acetylhexosaminidase (GM2)
  2. ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase (CMP-N-acetylneuraminate)
  3. a ganglioside GD3 (d18:1(4E)) + acetyl-CoA = a ganglioside Ac-O-7-GD3(d18:1(4E)) + CoA

  Catalysed by HEXA, HEXB, HEXD, ST8SIA1 and 1 morecytosol

• CMP-N-acetyl-9-O-acetyl-beta-neuraminateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol, nucleus

• CMP-N-acetyl-7-O-acetyl-beta-neuraminateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol, nucleus

• beta-D-galactoseReach from here →
  1. beta-1,3-galactosyltransferase (GM2)
  2. beta-galactosidase (GM1)
  3. galactose[n] = beta-D-galactose[n]

  Catalysed by B3GALT4, LCT, GLB1, GLB1L and 2 morecytosol, nucleusOne of 2 routes

• acetateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol, extracellular spaceOne of 2 routes

• formic acidReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol, extracellular spaceOne of 2 routes

• propionic acidReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol, extracellular spaceOne of 2 routes

• butyric acidReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol, extracellular spaceOne of 2 routes

• 3-hydroxypyruvic acidReach from here →
  1. exo-alpha-sialidase (GM2)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. L-Serine:pyruvate aminotransferase

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

• 3-oxopropanoateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol

• D-serineReach from here →
  1. exo-alpha-sialidase (GM2)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. D-serine = pyruvate + NH4(+)

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

• (R)-lactic acidReach from here →
  1. exo-alpha-sialidase (GM2)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (R)-Lactate:NAD+ oxidoreductase

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

• (S)-lactic acidReach from here →
  1. exo-alpha-sialidase (GM2)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (S)-Lactate:NAD+ oxidoreductase

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

• 2-iminopropanoateReach from here →
  1. exo-alpha-sialidase (GM2)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

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

• phosphoenolpyruvic acidReach from here →
  1. exo-alpha-sialidase (GM2)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. pyruvate kinase (dTDP)

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

• N-acetyl-D-glucosamineReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol

• an isogloboside iGb3Cer (d18:1(4E))Reach from here →
  1. beta-N-acetylhexosaminidase (GM2)
  2. exo-alpha-sialidase (GM3)
  3. a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) + UDP-alpha-D-galactose = an isogloboside iGb3Cer (d18:1(4E)) + UDP + H(+)

  Catalysed by HEXA, HEXB, HEXD, NEU4 and 4 morecytosol

• a 3-acylpyruvateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol

• 2-methyl-3-oxopropanoateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol

• L-methionineReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol

• L-alanineReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol, mitochondrial matrixOne of 18 routes

• alpha-D-galactose 1-phosphateReach from here →
  1. beta-1,3-galactosyltransferase (GM2)
  2. beta-galactosidase (GM1)
  3. galactokinase (galactose)

  Catalysed by B3GALT4, LCT, GLB1, GLB1L and 3 morecytosolOne of 2 routes

• aldehydo-N-acetyl-D-mannosamine 6-phosphateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosolOne of 2 routes

• a neolactoside IV(3)-alpha-NeuAc-nLc4Cer(d18:1(4E))Reach from here →
  1. exo-alpha-sialidase (GM2)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. ST3 beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)

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

• (3S)-citramalyl-CoAReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol

• 5-amino-2-oxopentanoateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol

• (4S)-4-hydroxy-2-oxoglutarateReach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol

• ganglioside GM1 (d18:1(4E)/18:0)Reach from here →
  1. exo-alpha-sialidase (GM2)
  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 morecytosol, nucleus

• beta-D-3-sulfogalactosyl-(1->4)-beta-D-glucosyl-(1<->1')-N-acylsphing-4-enineReach from here →
  1. beta-N-acetylhexosaminidase (GM2)
  2. exo-alpha-sialidase (GM3)
  3. a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E)) + 3'-phosphoadenylyl sulfate = beta-D-3-sulfogalactosyl-(1->4)-beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by HEXA, HEXB, HEXD, NEU4 and 4 morecytosol

• a ganglioside GT3 (d18:1(4E))Reach from here →
  1. beta-N-acetylhexosaminidase (GM2)
  2. ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase (CMP-N-acetylneuraminate)
  3. alpha-N-acetylneuraminate alpha-2,8-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by HEXA, HEXB, HEXD, ST8SIA1 and 1 morecytosolOne of 6 routes

• a ganglioside GT1a (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GM2)
  2. beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)
  3. ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by B3GALT4, ST3GAL1, ST3GAL4, ST3GAL2 and 1 morecytosolOne of 6 routes

• a ganglioside GT1b (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GM2)
  2. beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)
  3. ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by B3GALT4, ST3GAL1, ST3GAL4, ST3GAL2 and 1 morecytosolOne of 8 routes

• a ganglioside GQ1b (d18:1(4E))Reach from here →
  1. exo-alpha-sialidase (GM2)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 2 morecytosol, nucleusOne of 4 routes

• a ganglioside GM4 (d18:1(4E))Reach from here →
  1. exo-alpha-sialidase (GM2)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + CMP-N-acetyl-beta-neuraminate = a ganglioside GM4 (d18:1(4E)) + CMP + H(+)

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

• ganglioside GM2 (d18:1(4E)/18:0)Reach from here →
  1. exo-alpha-sialidase (GM2)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. ganglioside GA2 (d18:1(4E)/18:0) + CMP-N-acetyl-beta-neuraminate = ganglioside GM2 (d18:1(4E)/18:0) + CMP + H(+)

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

• a ganglioside GM1b (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GM2)
  2. a ganglioside GM1 (d18:1(4E)) + H2O = a ganglioside GA1 (d18:1(4E)) + N-acetylneuraminate
  3. a ganglioside GA1 (d18:1(4E)) + CMP-N-acetyl-beta-neuraminate = a ganglioside GM1b (d18:1(4E)) + CMP + H(+)

  Catalysed by B3GALT4, NEU3, ST3GAL1, ST3GAL4 and 1 morecytosolOne of 2 routes

• a ganglioside GD1alpha (d18:1(4E))Reach from here →
  1. exo-alpha-sialidase (GM2)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. a ganglioside GM1b (d18:1(4E)) + CMP-N-acetyl-beta-neuraminate = a ganglioside GD1alpha (d18:1(4E)) + CMP + H(+)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 5 morecytosol, nucleus

• a ganglioside GT1aalpha (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GM2)
  2. beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)
  3. a ganglioside GD1a (d18:1(4E)) + CMP-N-acetyl-beta-neuraminate = a ganglioside GT1aalpha (d18:1(4E)) + CMP + H(+)

  Catalysed by B3GALT4, ST3GAL1, ST3GAL4, ST3GAL2 and 1 morecytosolOne of 2 routes

• a ganglioside GQ1balpha (d18:1(4E))Reach from here →
  1. exo-alpha-sialidase (GM2)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. a ganglioside GT1b (d18:1(4E)) + CMP-N-acetyl-beta-neuraminate = a ganglioside GQ1balpha (d18:1(4E)) + CMP + H(+)

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

• a ganglioside GD3Reach from here →
  1. exo-alpha-sialidase (GM2)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. a ganglioside GM3 + CMP-N-acetyl-beta-neuraminate = a ganglioside GD3 + CMP + H(+)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 3 morecytosol, nucleus

• a ganglioside GT3Reach from here →
  1. exo-alpha-sialidase (GM2)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. a ganglioside GD3 + CMP-N-acetyl-beta-neuraminate = a ganglioside GT3 + CMP + H(+)

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

• a ganglioside GD1aReach from here →
  1. exo-alpha-sialidase (GM2)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. a ganglioside GM1 + CMP-N-acetyl-beta-neuraminate = a ganglioside GD1a + CMP + H(+)

  Catalysed by NEU4, NEU1, NEU3, NEU2 and 3 morecytosol, nucleus

• N-acetyl-alpha-neuraminosyl-(2->3)-beta-D-galactosyl-(1<->1')-ceramideReach from here →
  1. exo-alpha-sialidase (GM2)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. a beta-D-Gal-(1<->1')-ceramide + CMP-N-acetyl-beta-neuraminate = N-acetyl-alpha-neuraminosyl-(2->3)-beta-D-galactosyl-(1<->1')-ceramide + CMP + H(+)

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