an N-acylsphinganine

Three steps

• a beta-D-Gal-(1->4)-beta-D-Glc-(1<->1)-Cer(d18:1(4E))Reach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. UDP-glucose:N-acylsphingosine D-glucosyltransferase
  3. glucosylceramide pool to LacCer pool conversion

  Catalysed by DEGS1, DEGS2, UGCG, ALG1L2 and 34 morecytosol, endoplasmic reticulumOne of 5 routes

• 4-methylpentanalReach 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. 6 reduced [adrenodoxin] + cholesterol + 3 O2 + 6 H(+) = 4-methylpentanal + pregnenolone + 6 oxidized [adrenodoxin] + 4 H2O

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 1 morecytosol

• a diacylglycerolReach 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. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. a triacylglycerol + H2O = a diacylglycerol + a fatty acid + H(+)

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 22 morecytosolOne of 2 routes

• CHEBI:18132Reach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. Sphingomyelin cholinephosphohydrolase

  Catalysed by DEGS1, DEGS2, SGMS1, SGMS2 and 5 morecytosol, Golgi apparatusOne of 4 routes

• phosphatidyl-L-serineReach 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 2 routes

• cholesteryl 3-beta-D-galactosideReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. an N-acylsphing-4-enine + UDP-alpha-D-galactose = a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + UDP + H(+)
  3. a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-galactoside + an N-acylsphing-4-enine

  Catalysed by DEGS1, DEGS2, UGT8, GBA1 and 1 morecytosolOne of 3 routes

• cholesteryl 3-beta-D-xylosideReach 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 beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 1 morecytosolOne of 3 routes

• 11-cis-retinyl esterReach from here →
  1. an N-acylsphinganine + H2O = sphinganine + a fatty acid
  2. a fatty acid + ATP + CoA = a fatty acyl-CoA + AMP + diphosphate
  3. 11-cis-retinol + a fatty acyl-CoA = 11-cis-retinyl ester + CoA

  Catalysed by ACER2, ACER1, ACER3, ACSL3 and 1 more

• 13-cis-retinyl esterReach from here →
  1. an N-acylsphinganine + H2O = sphinganine + a fatty acid
  2. a fatty acid + ATP + CoA = a fatty acyl-CoA + AMP + diphosphate
  3. 13-cis-retinol + a fatty acyl-CoA = 13-cis-retinyl ester + CoA

  Catalysed by ACER2, ACER1, ACER3, ACSL3 and 1 more

• 9-cis-retinyl esterReach from here →
  1. an N-acylsphinganine + H2O = sphinganine + a fatty acid
  2. a fatty acid + ATP + CoA = a fatty acyl-CoA + AMP + diphosphate
  3. 9-cis-retinol + a fatty acyl-CoA = 9-cis-retinyl ester + CoA

  Catalysed by ACER2, ACER1, ACER3, ACSL3 and 1 more

• (5S)-hydroperoxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoate + O2 = (5S)-hydroperoxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoate

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

• (5Z)-dodecenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

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

• O-[(5Z),(8Z),(11Z),(14Z)-eicosatetraenoyl]-beta-D-glucuronateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + UDP-alpha-D-glucuronate = O-[(5Z),(8Z),(11Z),(14Z)-eicosatetraenoyl]-beta-D-glucuronate + UDP

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 morecytosol, extracellular space

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 15 morecytosolOne of 6 routes

• (7Z)-tetradecenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

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

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

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

• (7Z,10Z)-hexadecadienoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 morecytosol, mitochondrial matrix

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

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

• (5Z)-octenoyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

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

• a long-chain alcohol wax esterReach from here →
  1. an N-acylsphinganine + H2O = sphinganine + a fatty acid
  2. a fatty acid + ATP + CoA = a fatty acyl-CoA + AMP + diphosphate
  3. a long chain fatty alcohol + a fatty acyl-CoA = a long-chain alcohol wax ester + CoA

  Catalysed by ACER2, ACER1, ACER3, ACSL3 and 2 more

• N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-ethanolamineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. fatty acylamide amidohydrolase

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

• CHEBI:27540Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 13 morecytosolOne of 6 routes

• dodecanalReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. 2 H+[r] + lauric acid[r] + NADH[r] = H2O[r] + NAD+[r] + dodecanal[r]

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 morecytosol, extracellular space, endoplasmic reticulumOne of 3 routes

• alpha-D-galactoseReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. UDP-alpha-D-galactose:N-acylsphingosine D-galactosyltransferase
  3. D-galactosyl-N-acylsphingosine galactohydrolase

  Catalysed by DEGS1, DEGS2, UGT8, GALCcytosol, lysosome

• CHEBI:28701Reach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 10 morecytosol, peroxisomeOne of 7 routes

• phosphocholineReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. ceramide:phosphatidylcholine cholinephosphotransferase
  3. a sphingomyelin + H2O = phosphocholine + an N-acylsphing-4-enine + H(+)

  Catalysed by DEGS1, DEGS2, SGMS1, SGMS2 and 5 morecytosol, Golgi apparatusOne of 2 routes

• (24S)-hydroxycholesterolReach 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. cholesterol + reduced [NADPH--hemoprotein reductase] + O2 = (24S)-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 1 morecytosol

• a sterol esterReach 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 sterol + a 1,2-diacyl-sn-glycero-3-phosphocholine = a sterol ester + a 1-acyl-sn-glycero-3-phosphocholine

  Catalysed by SGMS2, SAMD8, SGMS1, LCAT

• 12-hydroxydodecanoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. alkane 1-monooxygenase (lauric acid)

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 morecytosol, extracellular spaceOne of 2 routes

• cholesteryl hexadecanoateReach 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. cholesterol + hexadecanoyl-CoA = cholesteryl hexadecanoate + CoA

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 1 morecytosol

• a 1-O-alkyl-2-acyl-sn-glycero-3-phosphocholineReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-alkyl-sn-glycero-3-phosphocholine O-acyltransferase

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 6 morecytosol, Golgi apparatus

• sphingosine 1-phosphateReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. ATP:sphingosine 1-phosphotransferase

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 5 morecytosol, Golgi apparatusOne of 2 routes

• D-galactoseReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. an N-acylsphing-4-enine + UDP-alpha-D-galactose = a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + UDP + H(+)
  3. a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + H2O = D-galactose + an N-acylsphing-4-enine

  Catalysed by DEGS1, DEGS2, UGT8, GBA1 and 3 morecytosol

• cholesteryl (9Z,12Z)-octadecadienoateReach 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. (9Z,12Z)-octadecadienoyl-CoA + cholesterol = cholesteryl (9Z,12Z)-octadecadienoate + CoA

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 1 morecytosolOne of 2 routes

• D-glucoseReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. UDP-glucose:N-acylsphingosine D-glucosyltransferase
  3. D-Glucosyl-N-acylsphingosine glucohydrolase

  Catalysed by DEGS1, DEGS2, UGCG, GBA1 and 2 morecytosolOne of 5 routes

• 25-hydroxycholesterolReach 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. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 1 morecytosolOne of 3 routes

• N-octanoylsphing-4-enineReach 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. beta-D-glucosyl-N-octanoylsphing-4E-enine + cholesterol = N-octanoylsphing-4-enine + cholesteryl 3-beta-D-glucoside

  Catalysed by DEGS1, DEGS2, GBA1, GBA2cytosol

• cholesteryl (9Z-octadecenoate)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. cholesterol + (9Z)-octadecenoyl-CoA = cholesteryl (9Z-octadecenoate) + CoA

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 2 morecytosolOne of 2 routes

• N-(acetyl)-sphing-4-enineReach from here →
  1. delta 4-desaturase, sphingolipid (dihydroceramide pool)
  2. acyl-CoA:sphingosine N-acyltransferase
  3. 1-O-hexadecyl-2-acetyl-sn-glycero-3-phosphocholine + sphing-4-enine = 1-O-hexadecyl-sn-glycero-3-phosphocholine + N-(acetyl)-sphing-4-enine + H(+)

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

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

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 4 morecytosol, Golgi apparatusOne of 6 routes

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

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 7 morecytosol, Golgi apparatusOne of 8 routes

• (24R)-hydroxycholesterolReach 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. cholesterol + reduced [NADPH--hemoprotein reductase] + O2 = (24R)-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

  Catalysed by DEGS1, DEGS2, GBA1, GBA2 and 1 morecytosol

• taurineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

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

• 2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-glycerolReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. fatty acid amide hydrolase (2-arachidonoylglycerol)

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

• 1,2,3-tri-(9Z-octadecenoyl)-glycerolReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. 1,2-di-(9Z-octadecenoyl)-glycerol + (9Z)-octadecenoate + H(+) = 1,2,3-tri-(9Z-octadecenoyl)-glycerol + H2O

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 6 morecytosol, extracellular space

• 16-hydroxyhexadecanoateReach from here →
  1. N-acylsphingosine amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. Fatty Acid Omega Oxidation (C16->W-Ohc16), Endoplasmatic Reticulum

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 3 morecytosol, extracellular space, endoplasmic reticulumOne of 2 routes

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

  Catalysed by DEGS1, DEGS2, ASAH1, ACER2 and 5 morecytosol, Golgi apparatusOne of 3 routes

• glycineReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. glycine N-acyltransferase (N-arachidonoylglycine)

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

• (R)-methylmalonyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

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

• (S)-methylmalonyl-CoAReach from here →
  1. N-acylsphingosine amidohydrolase
  2. sphingosine N-acyltransferase (sphinganine)
  3. (S)-methylmalonyl-CoA hydrolase

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