an N-acylsphinganine

Three steps

• 3'-dephospho-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 8 morecytosol, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 morecytosol, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 9 morecytosol, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 9 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + ATP + CoA = (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + AMP + diphosphate

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 12 morecytosol, extracellular space

• butanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 13 morecytosolOne of 9 routes

• dodecanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 13 routes

• glutaryl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Production of Glutaryl Carnitine

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 morecytosolOne of 2 routes

• eicosanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 19 routes

• itaconyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 morecytosol, mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 19 routes

• tetradecanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 16 routes

• octanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 8 morecytosol, mitochondrial matrixOne of 20 routes

• (9Z)-octadecenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (9Z)-octadecenoate + ATP + CoA = (9Z)-octadecenoyl-CoA + AMP + diphosphate

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 9 morecytosol, extracellular space

• phytanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. phytanate-CoA ligase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 morecytosolOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 14 morecytosolOne of 22 routes

• prostaglandin H2Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. prostaglandin-endoperoxide synthase (arachidonate)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 morecytosol, extracellular spaceOne of 3 routes

• (12S)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + O2 = (12S)-hydroperoxy-(5Z,8Z,10E,14Z)-eicosatetraenoate

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 morecytosol, extracellular space

• (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + O2 = (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 morecytosol, extracellular space

• (5S)-hydroperoxy-(6E,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + O2 = (5S)-hydroperoxy-(6E,8Z,11Z,14Z)-eicosatetraenoate

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 morecytosol, extracellular space

• leukotriene A4Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + O2 = leukotriene A4 + H2O

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 morecytosol, extracellular space

• (13S)-hydroperoxy-(9Z,11E)-octadecadienoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. Linoleate:oxygen 13-oxidoreductase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 morecytosol, extracellular space

• a 1-acylglycerone 3-phosphateReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. dihydroxyacetone phosphate + an acyl-CoA = a 1-acylglycerone 3-phosphate + CoA

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 3 morecytosol, Golgi apparatus

• (R)-2-hydroxyoctadecanoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. octadecanoate + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = (R)-2-hydroxyoctadecanoate + 2 Fe(III)-[cytochrome b5] + H2O

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 morecytosol, extracellular space

• ethanolamineReach from here →
  1. an N-acylsphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acylsphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. CDPethanolamine:1,2-diacylglycerol ethanolaminephosphotransferase
  3. L-1-phosphatidylethanolamine:L-serine phosphatidyltransferase

  Catalysed by SGMS2, SAMD8, SELENOI, CEPT1 and 1 morecytosolOne of 3 routes

• an N-acylglycineReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 5 morecytosol, Golgi apparatus

• spermidineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 9 morecytosolOne of 3 routes

• L-methionineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. N-acetylglutamate synthase (N-acetylmethionine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 8 morecytosol, mitochondrial matrix

• a 2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. an N-acylsphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acylsphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. an N-acylsphing-4-enine + a 1,2-diacyl-sn-glycero-3-phosphocholine = a sphingomyelin + a 1,2-diacyl-sn-glycerol
  3. a 2-acyl-sn-glycero-3-phosphocholine + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phosphocholine + CoA

  Catalysed by SGMS2, SAMD8, SGMS1, LPGAT1One of 13 routes

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol)Reach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. a 1-acyl-sn-glycero-3-phospho-(1D-myo-inositol) + an acyl-CoA = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol) + CoA

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 4 morecytosol, Golgi apparatusOne of 3 routes

• a 1-acyl-sn-glycero-3-phosphateReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sn-glycerol 3-phosphate + an acyl-CoA = a 1-acyl-sn-glycero-3-phosphate + CoA

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 6 morecytosol, Golgi apparatusOne of 2 routes

• L-alanineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. amino-acid N-acetyltransferase (N-acetyl-L-alanine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 morecytosol, mitochondrial matrix

• L-asparagineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. N-acetylglutamate synthase (N-acetyl-L-asparagine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 8 morecytosol, mitochondrial matrix

• L,L-cystathionineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 8 morecytosol, mitochondrial matrix

• a 1-acyl-sn-glycero-3-phosphocholineReach from here →
  1. an N-acylsphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acylsphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. an N-acylsphing-4-enine + a 1,2-diacyl-sn-glycero-3-phosphocholine = a sphingomyelin + a 1,2-diacyl-sn-glycerol
  3. a 1,2-diacyl-sn-glycero-3-phosphocholine + H2O = a 1-acyl-sn-glycero-3-phosphocholine + a fatty acid + H(+)

  Catalysed by SGMS2, SAMD8, SGMS1, PLA2G10 and 29 moreOne of 14 routes

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol 4-phosphate)Reach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol 4-phosphate)(out) + cholesterol(in) = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol 4-phosphate)(in) + cholesterol(out)

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 1 morecytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• phosphoethanolamineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. ATP:sphinganine 1-phosphotransferase
  3. sphinganine 1-phosphate = hexadecanal + phosphoethanolamine

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 5 morecytosolOne of 2 routes

• a CDP-1,2-diacyl-sn-glycerolReach from here →
  1. an N-acylsphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acylsphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. a 1,2-diacyl-sn-glycerol + ATP = a 1,2-diacyl-sn-glycero-3-phosphate + ADP + H(+)
  3. a 1,2-diacyl-sn-glycero-3-phosphate + CTP + H(+) = a CDP-1,2-diacyl-sn-glycerol + diphosphate

  Catalysed by SGMS2, SAMD8, DGKI, DGKA and 12 more

• a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol-4,5-bisphosphate)Reach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
  3. a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol-4,5-bisphosphate)(out) + cholesterol(in) = a 1,2-diacyl-sn-glycero-3-phospho-(1D-myo-inositol-4,5-bisphosphate)(in) + cholesterol(out)

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 2 morecytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• (3Z)-dodecenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 morecytosol, mitochondrial matrixOne of 3 routes

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 9 morecytosol, endoplasmic reticulumOne of 3 routes

• deoxycholoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 9 morecytosolOne of 4 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + H2O = (5Z,8Z,11Z,14Z)-eicosatetraenoate + glycine

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 morecytosol, extracellular space

• sphing-4-enine 1-phosphateReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. sphing-4-enine + ATP = sphing-4-enine 1-phosphate + ADP + H(+)

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 4 morecytosol, Golgi apparatus

• (9S)-hydroperoxy-(10E,12Z)-octadecadienoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. linoleate:oxygen 9-oxidoreductase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 morecytosol, extracellular space

• decanoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 13 morecytosolOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Carnitine O-Palmitoyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 morecytosolOne of 6 routes

• (9Z)-hexadecenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (palmitolate)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 17 routes

• a cardiolipinReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1'-[1,2-diacyl-sn-glycero-3-phospho],3'-[1-acyl-sn-glycero-3-phospho]-glycerol + an acyl-CoA = a cardiolipin + CoA

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 3 morecytosol, Golgi apparatusOne of 2 routes

• an N-acyl-1,2-diacyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. an N-acylsphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acylsphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. an N-acylsphing-4-enine + a 1,2-diacyl-sn-glycero-3-phosphocholine = a sphingomyelin + a 1,2-diacyl-sn-glycerol
  3. a 1,2-diacyl-sn-glycero-3-phosphoethanolamine + a 1,2-diacyl-sn-glycero-3-phosphocholine = an N-acyl-1,2-diacyl-sn-glycero-3-phosphoethanolamine + a 2-acyl-sn-glycero-3-phosphocholine + H(+)

  Catalysed by SGMS2, SAMD8, SGMS1, PLA2G4E and 2 moreOne of 4 routes