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

Three steps

• 2-iminopropanoateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by NEU3, NPL, RIDAextracellular space, cytosol

• phosphoenolpyruvic acidReach 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

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

  Catalysed by NEU3, NPL, RENBPextracellular space, cytosol

• taurineReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by HEXA, HEXB, SLC33A1, BAATlysosome, cytosolOne of 2 routes

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

  Catalysed by NEU3, NPL, FAHD1extracellular space, cytosol

• glycineReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. glycine N-acyltransferase (N-arachidonoylglycine)

  Catalysed by HEXA, HEXB, SLC33A1, GLYAT and 2 morelysosome, cytosol, mitochondrial matrixOne of 10 routes

• (R)-methylmalonyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by HEXA, HEXB, SLC33A1, ALDH6A1lysosome, cytosol, mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by HEXA, HEXB, SLC33A1lysosome, cytosol, mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by HEXA, HEXB, SLC33A1, ACP2lysosome, cytosolOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by HEXA, HEXB, SLC33A1lysosome, cytosol, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by HEXA, HEXB, SLC33A1, ACAA1 and 1 morelysosome, cytosol, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

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

• butanoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by HEXA, HEXB, SLC33A1, ACSM4 and 5 morelysosome, cytosolOne of 9 routes

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

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 9 routes

• glutaryl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Production of Glutaryl Carnitine

  Catalysed by HEXA, HEXB, SLC33A1, CPT1A and 2 morelysosome, cytosolOne of 2 routes

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

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 12 routes

• itaconyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by HEXA, HEXB, SLC33A1, SUCLG1 and 2 morelysosome, cytosol, mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 13 routes

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

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 11 routes

• octanoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by HEXA, HEXB, SLC33A1, CROTlysosome, cytosol, mitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. phytanate-CoA ligase

  Catalysed by HEXA, HEXB, SLC33A1lysosome, cytosolOne of 5 routes

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

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 6 morelysosome, cytosolOne of 15 routes

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  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 NEU3, NPL, MPSTextracellular space, cytosol

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

• sphing-4-enineReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by HEXA, HEXB, SLC33A1, ASAH1 and 3 morelysosome, cytosol, Golgi apparatusOne of 2 routes

• spermidineReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by HEXA, HEXB, SLC33A1, SAT1 and 1 morelysosome, cytosolOne of 3 routes

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

  Catalysed by HEXA, HEXB, SLC33A1, NAGSlysosome, cytosol, mitochondrial matrixOne of 2 routes

• L-alanineReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. amino-acid N-acetyltransferase (N-acetyl-L-alanine)

  Catalysed by HEXA, HEXB, SLC33A1lysosome, cytosol, mitochondrial matrixOne of 19 routes

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

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

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

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

• alpha-D-galactose 1-phosphateReach from here →
  1. beta-1,3-galactosyltransferase (GD2)
  2. Beta-Galactosidase, Lysosomal
  3. galactokinase (galactose)

  Catalysed by B3GALT4, GLB1, GALK1, GALK2cytosol, lysosomeOne of 2 routes

• an acyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by HEXA, HEXB, SLC33A1, ASAH1 and 3 morelysosome, cytosol, Golgi apparatusOne of 2 routes

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

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

• aldehydo-N-acetyl-D-mannosamine 6-phosphateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. ATP:N-acyl-D-mannosamine 6-phosphotransferase

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

• a neolactoside IV(3)-alpha-NeuAc-nLc4Cer(d18:1(4E))Reach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. ST3 beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by NEU3, CMAS, ST3GAL6extracellular space, nucleus, cytosolOne of 3 routes

• (3S)-citramalyl-CoAReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by NEU3, NPL, CLYBLextracellular space, cytosol

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 1 morelysosome, cytosol, endoplasmic reticulumOne of 3 routes

• 5-amino-2-oxopentanoateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by NEU3, NPL, AGXT2extracellular space, cytosol

• deoxycholoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 1 morelysosome, cytosolOne of 4 routes

• decanoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by HEXA, HEXB, SLC33A1, ACSM4 and 5 morelysosome, cytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by HEXA, HEXB, SLC33A1, CPT1A and 2 morelysosome, cytosolOne of 4 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 11 routes

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by NEU3, NPL, HOGA1extracellular space, cytosol

• 3-hydroxyisovaleryl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by HEXA, HEXB, SLC33A1, CPT1A and 1 morelysosome, cytosol

• CHEBI:63544Reach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

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

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

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

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

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 10 routes

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

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 9 routes

• (2E)-octadecenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by HEXA, HEXB, SLC33A1, CPT1A and 2 morelysosome, cytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

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