a ganglioside GD3 (d18:1(4E))

Three steps

• CHEBI:18095Reach from here →
  1. solute carrier (acetyl-CoA)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Transport of Hydroxy-Proline

  Catalysed by SLC33A1, SLC1A4cytosol, mitochondrial matrix, extracellular space

• O-octanoyl-(R)-carnitineReach from here →
  1. solute carrier (acetyl-CoA)
  2. Carnitine O-Palmitoyltransferase
  3. solute carrier (L-carnitine)

  Catalysed by SLC33A1, CPT1A, CPT1C, CPT1B and 1 morecytosol, mitochondrial matrixOne of 11 routes

• CHEBI:18132Reach from here →
  1. solute carrier (acetyl-CoA)
  2. Acetyl-CoA:choline O-acetyltransferase
  3. ATP:choline phosphotransferase

  Catalysed by SLC33A1, CHAT, CHKA, CHKBcytosol, nucleus

• dodecanoateReach from here →
  1. solute carrier (acetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. Lauroyl-CoA hydrolase

  Catalysed by SLC33A1, SLC27A2, ACSL4, ACSL3 and 9 morecytosolOne of 2 routes

• a globoside Gb3Cer (d18:1(4E))Reach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. exo-alpha-sialidase (GM3)
  3. alpha 1,4-galactosyltransferase (P blood group) (LacCer pool)

  Catalysed by NEU3, NEU4, NEU1, NEU2 and 1 moreextracellular space, cytosolOne of 3 routes

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. solute carrier (acetyl-CoA)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by SLC33A1, GLYAT, GLYATL2, GLYATL1 and 1 morecytosol, mitochondrial matrix

• D-glucosyl-N-acylsphingosineReach from here →
  1. Sialidase, Plasma Membrane (Neu3)
  2. exo-alpha-sialidase (GM3)
  3. beta-D-Galactosyl-1,4-beta-D-glucosylceramide galactohydrolase

  Catalysed by NEU3, NEU4, NEU1, NEU2 and 4 moreextracellular space, cytosol, lysosome

• N-(4-aminobutyl)-3-aminopropanalReach from here →
  1. solute carrier (acetyl-CoA)
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. primary-amine oxidase (spermidine)

  Catalysed by SLC33A1, SAT1, SAT2, AOC2 and 2 morecytosolOne of 3 routes

• (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoAReach from here →
  1. solute carrier (acetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (EPA)
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + O2 = (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoA + H2O2

  Catalysed by SLC33A1, SLC27A2, ACSL4, ACSL3 and 6 morecytosol

• 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. solute carrier (acetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. a 2-acyl-sn-glycero-3-phosphoethanolamine + octadecanoyl-CoA = 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by SLC33A1, SLC27A2, ACSL4, ACSL3 and 5 morecytosol

• ophthalmateReach from here →
  1. solute carrier (acetyl-CoA)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by SLC33A1, GLYAT, GLYATL2, GLYATL1 and 1 morecytosol, mitochondrial matrix

• S-methylmercury-L-cysteineReach from here →
  1. solute carrier (acetyl-CoA)
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. S-methylmercury-L-cysteine(out) + L-methionine(in) = S-methylmercury-L-cysteine(in) + L-methionine(out)

  Catalysed by SLC33A1, NAGS, SLC7A8cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-aminoisobutanoateReach from here →
  1. solute carrier (acetyl-CoA)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by SLC33A1, SLC7A10cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• 11-cis-retinyl esterReach from here →
  1. solute carrier (acetyl-CoA)
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol + a fatty acyl-CoA = 11-cis-retinyl ester + CoA

  Catalysed by SLC33A1, DGAT1, LRAT, AWAT1 and 2 morecytosol

• 5-(3,3-dimethylguanidino)-2-oxopentanoateReach from here →
  1. solute carrier (acetyl-CoA)
  2. ATP citrate lyase (citrate)
  3. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate

  Catalysed by SLC33A1, ACLY, AGXT2cytosolOne of 2 routes

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. solute carrier (acetyl-CoA)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by SLC33A1, GLYAT, GLYATL2, GLYATL1 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• 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

• 1-butanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. solute carrier (acetyl-CoA)
  2. Butanoate:CoA ligase (AMP-forming)
  3. 2-(9Z-octadecenoyl)-glycerol + butanoyl-CoA = 1-butanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SLC33A1, ACSM4, ACSM1, ACSM2A and 5 morecytosol

• 1-octanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. solute carrier (acetyl-CoA)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. 2-(9Z-octadecenoyl)-glycerol + octanoyl-CoA = 1-octanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SLC33A1, CROT, MOGAT2, MOGAT1cytosol, mitochondrial matrix

• 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. solute carrier (acetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + 2-(9Z-octadecenoyl)-glycerol = 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by SLC33A1, SLC27A2, ACSL4, ACSL3 and 7 morecytosol

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. solute carrier (acetyl-CoA)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by SLC33A1, GLYAT, GLYATL2, GLYATL1 and 1 morecytosol, mitochondrial matrixOne of 2 routes

• O-butanoyl-(R)-carnitineReach from here →
  1. solute carrier (acetyl-CoA)
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + (R)-carnitine = O-butanoyl-(R)-carnitine + CoA

  Catalysed by SLC33A1, ACSM4, ACSM1, ACSM2A and 4 morecytosol

• S-disulfanyl-L-cysteineReach from here →
  1. solute carrier (acetyl-CoA)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. S-sulfanyl-L-cysteine + L-cysteine = S-disulfanyl-L-cysteine + L-alanine

  Catalysed by SLC33A1, NAGS, CARS2cytosol, mitochondrial matrix

• 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

• 3-oxo-(9Z)-eicosenoyl-CoAReach from here →
  1. solute carrier (acetyl-CoA)
  2. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)
  3. very-long-chain 3-oxoacyl-CoA synthase ((7Z)-octadecenoyl-CoA)

  Catalysed by SLC33A1, CPT1A, CPT1C, CPT1B and 7 morecytosol

• (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoAReach from here →
  1. solute carrier (acetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (gamma-linolenate)
  3. (6Z,9Z,12Z)-octadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by SLC33A1, SLC27A2, ACSL4, ACSL3 and 8 morecytosolOne of 2 routes

• (2E,7Z,10Z)-hexadecatrienoyl-CoAReach from here →
  1. solute carrier (acetyl-CoA)
  2. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial
  3. (7Z,10Z)-hexadecadienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z)-hexadecatrienoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by SLC33A1, HADHA, ACADVL, HADHB and 2 morecytosol, mitochondrial matrix

• (2E,4Z,7Z,10Z,13Z)-hexadecapentaenoyl-CoAReach from here →
  1. solute carrier (acetyl-CoA)
  2. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial
  3. Fatty Acid Beta Oxidation (C16:4->C16:5), Mitochondrial

  Catalysed by SLC33A1, HADHA, ACADVL, HADHBcytosol, mitochondrial matrixOne of 4 routes

• (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. solute carrier (acetyl-CoA)
  2. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial
  3. (7Z,10Z,13Z)-hexadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by SLC33A1, HADHA, ACADVL, HADHB and 2 morecytosol, mitochondrial matrix

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

  Catalysed by SLC33A1, ECHS1, ACAA2, HADH and 1 morecytosol, mitochondrial matrixOne of 3 routes

• docosanoateReach from here →
  1. solute carrier (acetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (behenic acid)
  3. Docosanoyl-CoA hydrolase

  Catalysed by SLC33A1, SLC27A2, ACSL4, ACSL3 and 7 morecytosolOne of 4 routes

• CHEBI:240943Reach from here →
  1. solute carrier (acetyl-CoA)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by SLC33A1, GLYAT, GLYATL2, GLYATL1cytosol, mitochondrial matrixOne of 2 routes

• octadecanoateReach from here →
  1. solute carrier (acetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. Stearoyl-CoA hydrolase

  Catalysed by SLC33A1, SLC27A2, ACSL4, ACSL3 and 9 morecytosolOne of 4 routes

• octanoateReach from here →
  1. solute carrier (acetyl-CoA)
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. Octanoyl-CoA hydrolase

  Catalysed by SLC33A1, CROT, ACOT7cytosol, mitochondrial matrixOne of 2 routes

• alpha-linolenic acidReach from here →
  1. solute carrier (acetyl-CoA)
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by SLC33A1, CPT1A, CPT1C, CPT1Bcytosol, endoplasmic reticulumOne of 2 routes

• phenylacetylglycineReach from here →
  1. solute carrier (acetyl-CoA)
  2. glycine N-acyltransferase (N-arachidonoylglycine)
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

  Catalysed by SLC33A1, GLYAT, GLYATL2, GLYATL1cytosol, mitochondrial matrixOne of 6 routes

• decanoateReach from here →
  1. solute carrier (acetyl-CoA)
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. Decanoyl-CoA hydrolase

  Catalysed by SLC33A1, ACSM4, ACSM1, ACSM2A and 4 morecytosolOne of 2 routes

• a ganglioside GA2 (d18:1(4E))Reach from here →
  1. beta-1,4-N-acetyl-galactosaminyltransferase (GD3)
  2. Sialidase, Plasma Membrane (Neu3)
  3. exo-alpha-sialidase (GM2)

  Catalysed by B4GALNT1, NEU3, NEU4, NEU1 and 1 morecytosol, extracellular spaceOne of 5 routes

• alpha-D-galactoseReach from here →
  1. beta-1,4-N-acetyl-galactosaminyltransferase (GD3)
  2. beta-1,3-galactosyltransferase (GD2)
  3. Beta-Galactosidase, Lysosomal

  Catalysed by B4GALNT1, B3GALT4, GLB1cytosol, lysosomeOne of 2 routes

• all-cis-docosa-4,7,10,13,16,19-hexaenoic acidReach from here →
  1. solute carrier (acetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (DHA)
  3. (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA hydrolase

  Catalysed by SLC33A1, SLC27A2, ACSL4, ACSL3 and 7 morecytosolOne of 2 routes

• hexacosan-1-olReach from here →
  1. solute carrier (acetyl-CoA)
  2. long-chain-fatty-acid-CoA ligase (cerotic acid)
  3. hexacosanoyl-CoA + 2 NADPH + 2 H(+) = hexacosan-1-ol + 2 NADP(+) + CoA

  Catalysed by SLC33A1, SLC27A2, ACSL4, ACSL3 and 6 morecytosol

• N-acetyl-beta-D-galactosamineReach from here →
  1. beta-1,4-N-acetyl-galactosaminyltransferase (GD3)
  2. Sialidase, Plasma Membrane (Neu3)
  3. a ganglioside GM2 (d18:1(4E)) + H2O = a ganglioside GM3 (d18:1(4E)) + N-acetyl-beta-D-galactosamine

  Catalysed by B4GALNT1, NEU3, HEXA, HEXBcytosol, extracellular space

• CHEBI:28666Reach from here →
  1. solute carrier (acetyl-CoA)
  2. N-acetyl-LTE4 to leukotriene E4 conversion
  3. LTD4 hydrolysis

  Catalysed by SLC33A1, ZDHHC11B, NAA10, ESCO2 and 29 morecytosolOne of 2 routes

• O-decanoyl-(R)-carnitineReach from here →
  1. solute carrier (acetyl-CoA)
  2. medium-chain acyl-CoA ligase (decanoic acid)
  3. decanoyl-CoA + (R)-carnitine = O-decanoyl-(R)-carnitine + CoA

  Catalysed by SLC33A1, ACSM4, ACSM1, ACSM2A and 5 morecytosol

• pyridoxine 5'-phosphateReach from here →
  1. solute carrier (acetyl-CoA)
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by SLC33A1, SCLYcytosol, mitochondrial matrix

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

  Catalysed by SLC33A1, NAGScytosol, mitochondrial matrix