an N-acyl-(4R)-4-hydroxysphinganine

Three steps

• all-trans-retinolReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + H2O = (4R)-hydroxysphinganine + a fatty acid
  2. an all-trans-retinyl ester + H2O = 11-cis-retinol + a fatty acid + H(+)
  3. carboxylesterase (retinyl-ester)

  Catalysed by ACER3, RPE65, CES2, RBP4 and 18 morecytosolOne of 2 routes

• 1-O-acyl-sn-glycero-3-phosphocholine(1+)Reach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. diacylglycerol cholinephosphotransferase (1,2-diacylglycerol-LD-PC pool)
  3. phosphatidylcholine 2-acylhydrolase

  Catalysed by SGMS2, SAMD8, CHPT1, CEPT1 and 18 morecytosol, endoplasmic reticulumOne of 8 routes

• O-phosphoethanolamineReach from here →
  1. Phytoceramide amidohydrolase
  2. Sphingosine Kinase 1
  3. Sphingosine-1-Phosphate Lyase 1 (Phytosphingosine-1-Phosphate, Cytosol)

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

• L-cysteineReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 4 morecytosol, mitochondrial matrix

• hexadecanalReach from here →
  1. Phytoceramide amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. 2 H+[c] + NADH[c] + palmitate[c] = H2O[c] + hexadecanal[c] + NAD+[c]

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 morecytosol, extracellular spaceOne of 2 routes

• a sphingomyelinReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. diacylglycerol cholinephosphotransferase (1,2-diacylglycerol-LD-PC pool)
  3. ceramide:phosphatidylcholine cholinephosphotransferase

  Catalysed by SGMS2, SAMD8, CHPT1, CEPT1 and 1 morecytosol, Golgi apparatusOne of 13 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 6 morecytosol, mitochondrial matrix

• glycerolReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + H2O = (4R)-hydroxysphinganine + a fatty acid
  2. a monoacylglycerol + H2O = glycerol + a fatty acid + H(+)
  3. a monoacylglycerol + H2O = glycerol + a fatty acid + H(+)

  Catalysed by ACER3, LIPEOne of 2 routes

• CHEBI:17927Reach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 3 morecytosol

• a diacylglycerolReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-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

• phosphatidyl-L-serineReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-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

• D-glucosyl-N-acylsphingosineReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-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. UDP-glucose:N-acylsphingosine D-glucosyltransferase

  Catalysed by SGMS2, SAMD8, SGMS1, UGCGcytosolOne of 2 routes

• a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enineReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-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. an N-acylsphing-4-enine + UDP-alpha-D-galactose = a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + UDP + H(+)

  Catalysed by SGMS2, SAMD8, SGMS1, UGT8One of 2 routes

• a beta-D-xylosyl-(1<->1')-N-acylsphing-4-enineReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-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 beta-D-xylosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-xyloside + an N-acylsphing-4-enine

  Catalysed by SGMS2, SAMD8, SGMS1, GBA1 and 1 moreOne of 2 routes

• 11-cis-retinyl esterReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + H2O = (4R)-hydroxysphinganine + 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 ACER3, ACSL3, AWAT2

• 13-cis-retinyl esterReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + H2O = (4R)-hydroxysphinganine + 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 ACER3, ACSL3, AWAT2

• 9-cis-retinyl esterReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + H2O = (4R)-hydroxysphinganine + 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 ACER3, ACSL3, AWAT2

• (5S)-hydroperoxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoateReach from here →
  1. Phytoceramide 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

• a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enineReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-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. an N-acylsphing-4-enine + UDP-alpha-D-glucose = a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + UDP + H(+)

  Catalysed by SGMS2, SAMD8, SGMS1, UGCGOne of 2 routes

• (5Z)-dodecenoyl-CoAReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 6 morecytosol, mitochondrial matrixOne of 2 routes

• O-[(5Z),(8Z),(11Z),(14Z)-eicosatetraenoyl]-beta-D-glucuronateReach from here →
  1. Phytoceramide 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. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 11 morecytosolOne of 5 routes

• (7Z)-tetradecenoyl-CoAReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 11 morecytosolOne of 8 routes

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 6 morecytosol, mitochondrial matrixOne of 3 routes

• (7Z,10Z)-hexadecadienoyl-CoAReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 6 morecytosol, mitochondrial matrix

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 6 morecytosol, mitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 6 morecytosol, mitochondrial matrixOne of 2 routes

• a long-chain alcohol wax esterReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + H2O = (4R)-hydroxysphinganine + 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 ACER3, ACSL3, AWAT1, AWAT2

• N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-ethanolamineReach from here →
  1. Phytoceramide 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. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 9 morecytosolOne of 6 routes

• dodecanalReach from here →
  1. Phytoceramide 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

• CHEBI:28701Reach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 6 morecytosol, peroxisomeOne of 7 routes

• phosphocholineReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-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 = phosphocholine + a 1,2-diacyl-sn-glycerol + H(+)

  Catalysed by SGMS2, SAMD8, SGMS1, SMPD1

• a sterol esterReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-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. Phytoceramide 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

• a 1,3-diacylglycerolReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. a triacylglycerol + H2O = a 1,3-diacylglycerol + a fatty acid + H(+)

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 5 morecytosolOne of 5 routes

• a 1,2-diacylglycerolReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. a triacylglycerol + H2O = a 1,2-diacylglycerol + a fatty acid + H(+)

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 7 morecytosolOne of 6 routes

• taurineReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 4 morecytosolOne of 2 routes

• 2-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-glycerolReach from here →
  1. Phytoceramide 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. Phytoceramide 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. Phytoceramide 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. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-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 + L-serine = a 1,2-diacyl-sn-glycero-3-phospho-L-serine + choline

  Catalysed by SGMS2, SAMD8, SGMS1, PTDSS1One of 2 routes

• glycineReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. glycine N-acyltransferase (N-arachidonoylglycine)

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 6 morecytosol, mitochondrial matrixOne of 10 routes

• (R)-methylmalonyl-CoAReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 4 morecytosol, mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 3 morecytosol, mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 4 morecytosol, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 3 morecytosol, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. an N-acyl-(4R)-4-hydroxysphinganine + a 1,2-diacyl-sn-glycero-3-phosphoethanolamine = an N-acyl-(4R)-4-hydroxysphinganine-1-phosphoethanolamine + a 1,2-diacyl-sn-glycerol
  2. acyl-CoA:1,2-diacyl-sn-glycerol O-acyltransferase
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by SGMS2, SAMD8, DGAT1, MOGAT2 and 5 morecytosol, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. Phytoceramide amidohydrolase
  2. (10Z)-heptadecenoic acid formation
  3. Long-Chain-Fatty-Acid-Coa Ligase

  Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 8 morecytosol, extracellular space, endoplasmic reticulumOne of 8 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoAReach from here →
  1. Phytoceramide 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