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

Three steps

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

  Catalysed by NEU3, NPL, HOGA1extracellular space, cytosol

• ganglioside GM1 (d18:1(4E)/18:0)Reach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  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 NEU3, CMAS, ST3GAL5extracellular space, nucleus

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (EPA)

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

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 6 routes

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

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

• (13Z)-docosenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

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

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

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

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

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

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

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

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (DHA)

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

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

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 6 routes

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

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

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

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

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

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2 and 7 morelysosome, cytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

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

• dodecanedioyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Activation of dodecanedioic acid

  Catalysed by HEXA, HEXB, SLC33A1lysosome, cytosolOne of 4 routes

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

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

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

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

• hexanedioyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by HEXA, HEXB, SLC33A1lysosome, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

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

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

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

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by HEXA, HEXB, SLC33A1, CPT1A and 2 morelysosome, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

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

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by HEXA, HEXB, SLC33A1, CRAT and 1 morelysosome, cytosol, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by HEXA, HEXB, SLC33A1, ACAA1 and 3 morelysosome, cytosol, peroxisome

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

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

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

  Catalysed by HEXA, HEXB, SLC33A1, SLC27A2lysosome, cytosolOne of 2 routes

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

  Catalysed by HEXA, HEXB, SLC33A1, ACSM4 and 5 morelysosome, cytosolOne of 3 routes

• a ganglioside GD1a (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GD2)
  2. Sialidase, Cytosol (Neu2)
  3. beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by B3GALT4, NEU2, ST3GAL1, ST3GAL4 and 1 morecytosolOne of 7 routes

• a ganglioside GT1a (d18:1(4E))Reach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. ST8 alpha-N-acetyl-neuraminide alpha-2,8-sialyltransferase (CMP-N-acetylneuraminate)

  Catalysed by NEU3, CMAS, ST8SIA5extracellular space, nucleus, cytosolOne of 3 routes

• a ganglioside GQ1b (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GD2)
  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 7 routes

• a ganglioside GM4 (d18:1(4E))Reach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  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 NEU3, CMAS, ST3GAL5extracellular space, nucleus

• ganglioside GM2 (d18:1(4E)/18:0)Reach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  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 NEU3, CMAS, ST3GAL5extracellular space, nucleus

• a ganglioside GM1b (d18:1(4E))Reach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. a ganglioside GA1 (d18:1(4E)) + CMP-N-acetyl-beta-neuraminate = a ganglioside GM1b (d18:1(4E)) + CMP + H(+)

  Catalysed by NEU3, CMAS, ST3GAL1, ST3GAL4 and 1 moreextracellular space, nucleus

• a ganglioside GD1alpha (d18:1(4E))Reach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  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 NEU3, CMAS, ST6GALNAC3, ST6GALNAC6 and 2 moreextracellular space, nucleus

• a ganglioside GT1aalpha (d18:1(4E))Reach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. a ganglioside GD1a (d18:1(4E)) + CMP-N-acetyl-beta-neuraminate = a ganglioside GT1aalpha (d18:1(4E)) + CMP + H(+)

  Catalysed by NEU3, CMAS, ST6GALNAC6extracellular space, nucleus

• a ganglioside GQ1balpha (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GD2)
  2. beta-galactoside alpha-2,3-sialyltransferase (CMP-N-acetylneuraminate)
  3. a ganglioside GT1b (d18:1(4E)) + CMP-N-acetyl-beta-neuraminate = a ganglioside GQ1balpha (d18:1(4E)) + CMP + H(+)

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

• a ganglioside Fuc-GM1 (d18:1(4E))Reach from here →
  1. beta-1,3-galactosyltransferase (GD2)
  2. Sialidase, Cytosol (Neu2)
  3. a ganglioside GM1 (d18:1(4E)) + GDP-beta-L-fucose = a ganglioside Fuc-GM1 (d18:1(4E)) + GDP + H(+)

  Catalysed by B3GALT4, NEU2, FUT2cytosol

• a ganglioside GD3Reach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. a ganglioside GM3 + CMP-N-acetyl-beta-neuraminate = a ganglioside GD3 + CMP + H(+)

  Catalysed by NEU3, CMAS, ST8SIA3, ST8SIA6extracellular space, nucleus

• a ganglioside GT3Reach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. a ganglioside GD3 + CMP-N-acetyl-beta-neuraminate = a ganglioside GT3 + CMP + H(+)

  Catalysed by NEU3, CMAS, ST8SIA1extracellular space, nucleus

• a ganglioside GD1aReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. a ganglioside GM1 + CMP-N-acetyl-beta-neuraminate = a ganglioside GD1a + CMP + H(+)

  Catalysed by NEU3, CMAS, ST3GAL1, ST3GAL2extracellular space, nucleus

• N-acetyl-alpha-neuraminosyl-(2->3)-beta-D-galactosyl-(1<->1')-ceramideReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  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 NEU3, CMAS, ST3GAL5extracellular space, nucleus

• a ganglioside GT1bReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. CTP:N-acetylneuraminate cytidylyltransferase
  3. a ganglioside GD1b + CMP-N-acetyl-beta-neuraminate = a ganglioside GT1b + CMP + H(+)

  Catalysed by NEU3, CMAS, ST3GAL1, ST3GAL2extracellular space, nucleus

• (5Z)-tetradecenoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. long-chain-fatty-acid-CoA ligase (physeteric acid)

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

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

  Catalysed by HEXA, HEXB, SLC33A1, ACADM and 3 morelysosome, cytosol, mitochondrial matrix

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. Hexosaminidase A, Lysosomal
  2. solute carrier (acetyl-CoA)
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

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