a ganglioside GD2 (d18:1(4E))

Three steps

• CHEBI:15650Reach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by HEXA, HEXB, SLC33A1, ZDHHC11B and 31 morelysosome, cytosolOne of 2 routes

• CHEBI:15727Reach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)

  Catalysed by HEXA, HEXB, SLC33A1, NAA10lysosome, cytosol

• CHEBI:15746Reach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by HEXA, HEXB, SLC33A1, SAT1 and 1 morelysosome, cytosolOne of 2 routes

• 2-methyl-3-oxopropanoic acidReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by NEU3, NPL, AGXT2extracellular space, cytosol, mitochondrial matrix

• 11-cis-retinolReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by HEXA, HEXB, SLC33A1, DGAT1 and 4 morelysosome, cytosol

• oxaloacetateReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. ATP citrate lyase (citrate)

  Catalysed by HEXA, HEXB, SLC33A1, ACLYlysosome, cytosolOne of 4 routes

• (S)-lactateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)

  Catalysed by NEU3, NPL, LDHA, LDHB and 3 moreextracellular space, cytosol

• 2-oxobutanoateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate

  Catalysed by NEU3, NPL, AGXT2extracellular space, cytosol

• 2-oxoglutaramateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by NEU3, NPL, KYAT1extracellular space, cytosolOne of 2 routes

• galactitolReach from here →
  1. beta-1,3-galactosyltransferase (GD2)
  2. Beta-Galactosidase, Lysosomal
  3. galactitol:NADP+ 1-oxidoreductase

  Catalysed by B3GALT4, GLB1, AKR1B1cytosol, lysosome

• 3-fumarylpyruvateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by NEU3, NPL, FAHD1extracellular space, cytosol

• (R)-3-hydroxybutyric acidReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. transport of (R)-3-hydroxybutanoate and pyruvate (cytosol to extracellular)

  Catalysed by NEU3, NPL, SLC16A4, SLC16A5 and 4 moreextracellular space, cytosolOne of 2 routes

• 3-hydroxypyruvateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. L-serine + pyruvate = 3-hydroxypyruvate + L-alanine

  Catalysed by NEU3, NPL, AGXTextracellular space, cytosol

• anilineReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by HEXA, HEXB, SLC33A1, NAT2 and 1 morelysosome, cytosol

• aceneuramateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by NEU3, NPLextracellular space, cytosolOne of 2 routes

• CHEBI:17312Reach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by HEXA, HEXB, SLC33A1lysosome, cytosolOne of 2 routes

• 5'-deoxyadenosineReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  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 NEU3, NPL, TYW1B, TYW1extracellular space, cytosol

• L-cysteineReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

  Catalysed by HEXA, HEXB, SLC33A1, NAGSlysosome, cytosol, mitochondrial matrix

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by HEXA, HEXB, SLC33A1, HADHA and 2 morelysosome, cytosol, mitochondrial matrix

• CHEBI:17927Reach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by HEXA, HEXB, SLC33A1lysosome, cytosol

• a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GD2)
  2. Beta-Galactosidase, Lysosomal
  3. beta-galactosidase (galactose)

  Catalysed by B3GALT4, GLB1, LCT, GLB1Lcytosol, lysosome, extracellular spaceOne of 5 routes

• dTDPReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. pyruvate kinase (dTDP)

  Catalysed by NEU3, NPL, PKM, PKLRextracellular space, cytosol

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  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 NEU3, NPL, AGXT2extracellular space, cytosol

• 5-(3,3'-dimethylguanidino)-2-oxopentanoateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  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 NEU3, NPL, AGXT2extracellular space, cytosol

• 5-(3-methylguanidino)-2-oxopentanoateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  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 NEU3, NPL, AGXT2extracellular space, cytosol

• an alpha-Neu5Ac-(2->8)-alpha-Neu5Ac-(2->3)-beta-D-Gal-(1->4)-beta-D-GlcNAc derivativeReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  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 NEU3, CMAS, ST8SIA3extracellular space, nucleus

• alpha-Neu5Ac-(2->8)-alpha-Neu5Ac-(2->3)-beta-D-Gal-(1->4)-6S-D-GlcNAcReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  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 NEU3, CMAS, ST8SIA3extracellular space, nucleus

• CMP-N-acetyl-9-O-acetyl-beta-neuraminateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. CMP-N-acetyl-beta-neuraminate + acetyl-CoA = CMP-N-acetyl-9-O-acetyl-beta-neuraminate + CoA

  Catalysed by NEU3, CMAS, CASD1extracellular space, nucleus

• CMP-N-acetyl-7-O-acetyl-beta-neuraminateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. CMP-N-acetyl-beta-neuraminate + acetyl-CoA = CMP-N-acetyl-7-O-acetyl-beta-neuraminate + CoA

  Catalysed by NEU3, CMAS, CASD1extracellular space, nucleus

• (5Z)-dodecenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by HEXA, HEXB, SLC33A1, HADHA and 2 morelysosome, cytosol, mitochondrial matrixOne of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 5 routes

• (7Z)-tetradecenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 8 routes

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

  Catalysed by HEXA, HEXB, SLC33A1, HADHA and 2 morelysosome, cytosol, mitochondrial matrixOne of 3 routes

• (7Z,10Z)-hexadecadienoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by HEXA, HEXB, SLC33A1, HADHA and 2 morelysosome, cytosol, mitochondrial matrix

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by HEXA, HEXB, SLC33A1, ECHS1 and 2 morelysosome, cytosol, mitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by HEXA, HEXB, SLC33A1, ECHS1 and 2 morelysosome, cytosol, mitochondrial matrixOne of 2 routes

• CHEBI:27540Reach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by HEXA, HEXB, SLC33A1, ACSM4 and 5 morelysosome, cytosolOne of 6 routes

• beta-D-galactoseReach from here →
  1. beta-1,3-galactosyltransferase (GD2)
  2. Beta-Galactosidase, Lysosomal
  3. galactose[n] = beta-D-galactose[n]

  Catalysed by B3GALT4, GLB1, GALMcytosol, lysosome, nucleusOne of 2 routes

• a ganglioside GA1 (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GD2)
  2. Sialidase, Cytosol (Neu2)
  3. a ganglioside GM1 (d18:1(4E)) + H2O = a ganglioside GA1 (d18:1(4E)) + N-acetylneuraminate

  Catalysed by B3GALT4, NEU2, NEU3cytosolOne of 2 routes

• elaidic acidReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (elaidate)

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 5 routes

• CHEBI:28701Reach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by HEXA, HEXB, SLC33A1, ACAA1 and 2 morelysosome, cytosol, peroxisomeOne of 7 routes

• acetateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. transport of acetate and pyruvate (cytosol to extracellular)

  Catalysed by NEU3, NPL, SLC16A4, SLC16A5 and 3 moreextracellular space, cytosolOne of 2 routes

• formic acidReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. transport of formate and pyruvate (cytosol to extracellular)

  Catalysed by NEU3, NPL, SLC16A4, SLC16A5 and 3 moreextracellular space, cytosolOne of 2 routes

• propionic acidReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. transport of propanoate and pyruvate (cytosol to extracellular)

  Catalysed by NEU3, NPL, SLC16A4, SLC16A5 and 4 moreextracellular space, cytosolOne of 2 routes

• butyric acidReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. transport of butyrate and pyruvate (cytosol to extracellular)

  Catalysed by NEU3, NPL, SLC16A4, SLC16A5 and 4 moreextracellular space, cytosolOne of 2 routes

• 3-hydroxypyruvic acidReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. L-Serine:pyruvate aminotransferase

  Catalysed by NEU3, NPL, AGXTextracellular space, cytosolOne of 2 routes

• 3-oxopropanoateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate

  Catalysed by NEU3, NPL, AGXT2extracellular space, cytosol

• D-serineReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. D-serine = pyruvate + NH4(+)

  Catalysed by NEU3, NPL, SRRextracellular space, cytosol

• (R)-lactic acidReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (R)-Lactate:NAD+ oxidoreductase

  Catalysed by NEU3, NPL, LDHDextracellular space, cytosol

• (S)-lactic acidReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (S)-Lactate:NAD+ oxidoreductase

  Catalysed by NEU3, NPL, LDHB, LDHAL6Bextracellular space, cytosol, mitochondrial matrixOne of 5 routes