an N-acylsphing-4-enine

1. acyl-CoA:sphingosine N-acyltransferase
2. acyl-CoA pool formation and breakdown

Catalysed by ASAH1, ACER2, ACER1, ASAH2Golgi apparatus, cytosol

1. acyl-CoA:sphingosine N-acyltransferase
2. acyl-CoA pool formation and breakdown

Catalysed by ASAH1, ACER2, ACER1, ASAH2Golgi apparatus, cytosol

1. acyl-CoA:sphingosine N-acyltransferase
2. acyl-CoA pool formation and breakdown

Catalysed by ASAH1, ACER2, ACER1, ASAH2Golgi apparatus, cytosol

1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
2. 2 reduced [adrenodoxin] + cholesterol + O2 + 2 H(+) = (25R)-cholest-5-ene-3beta,26-diol + 2 oxidized [adrenodoxin] + H2O

Catalysed by GBA1, GBA2, CYP27A1

1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
2. 1-octadecanoyl-2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phospho-1D-myo-inositol 4,5-bisphosphate(out) + cholesterol(in) = 1-octadecanoyl-2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phospho-1D-myo-inositol 4,5-bisphosphate(in) + cholesterol(out)

Catalysed by GBA1, GBA2, OSBPL2inner side of the membrane, outer side of the membraneOne of 2 routes

1. N-Acylsphingosine amidohydrolase
2. (10Z)-heptadecenoic acid formation

Catalysed by ASAH1lysosome, extracellular spaceOne of 12 routes

1. N-Acylsphingosine amidohydrolase
2. (10Z)-heptadecenoic acid formation

Catalysed by ASAH1lysosome, extracellular spaceOne of 12 routes

1. N-Acylsphingosine amidohydrolase
2. (10Z)-heptadecenoic acid formation

Catalysed by ASAH1lysosome, extracellular spaceOne of 12 routes

1. N-Acylsphingosine amidohydrolase
2. (10Z)-heptadecenoic acid formation

Catalysed by ASAH1lysosome, extracellular spaceOne of 12 routes

1. acyl-CoA:sphingosine N-acyltransferase
2. a 3-acyl-sn-glycerol + an acyl-CoA = a 1,3-diacyl-sn-glycerol + CoA

Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moreGolgi apparatusOne of 3 routes

1. acyl-CoA:sphingosine N-acyltransferase
2. a 1-O-(1Z-alkenyl)-sn-glycero-3-phosphocholine + an acyl-CoA = a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphocholine + CoA

Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 moreGolgi apparatus

1. acyl-CoA:sphingosine N-acyltransferase
2. a 1-O-(1Z-alkenyl)-sn-glycero-3-phosphoethanolamine + an acyl-CoA = a 1-O-(1Z-alkenyl)-2-acyl-sn-glycero-3-phosphoethanolamine + CoA

Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moreGolgi apparatus

1. an N-acylsphing-4-enine + H2O = sphing-4-enine + a fatty acid
2. a fatty acid + ATP + CoA = a fatty acyl-CoA + AMP + diphosphate

Catalysed by NAAA, ASAH1, ACER2, ACER1 and 3 more

1. acyl-CoA:sphingosine N-acyltransferase
2. N-(9Z-octadecenoyl)-sphing-4-enine + H2O = sphing-4-enine + (9Z)-octadecenoate

Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moreGolgi apparatusOne of 3 routes

1. N-Acylsphingosine amidohydrolase
2. (10Z)-heptadecenoic acid formation

Catalysed by ASAH1lysosome, extracellular spaceOne of 12 routes

1. ceramide:phosphatidylcholine cholinephosphotransferase
2. 1-(9Z-octadecenoyl)-2-acyl-sn-3-glycerol + a sphingomyelin = a 1-(9Z-octadecenoyl)-2-acyl-sn-glycero-3-phosphocholine + an N-acylsphing-4-enine

Catalysed by SGMS1, SGMS2Golgi apparatusOne of 2 routes

1. an N-acylsphing-4-enine + UDP-alpha-D-galactose = a beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine + UDP + H(+)
2. 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 UGT8, ST3GAL5

1. N-Acylsphingosine amidohydrolase
2. (10Z)-heptadecenoic acid formation

Catalysed by ASAH1lysosome, extracellular spaceOne of 12 routes

1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
2. octadecanoyl-CoA + cholesterol = cholesteryl octadecanoate + CoA

Catalysed by GBA1, GBA2, SOAT1

1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
2. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + cholesterol = cholesteryl (5Z,8Z,11Z,14Z)-eicosatetraenoate + CoA

Catalysed by GBA1, GBA2, SOAT2, SOAT1One of 2 routes

1. acyl-CoA:sphingosine N-acyltransferase
2. sphing-4-enine + 2 Fe(II)-[cytochrome b5] + O2 + 2 H(+) = sphinga-4E,14Z-dienine + 2 Fe(III)-[cytochrome b5] + 2 H2O

Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 1 moreGolgi apparatus

1. an N-acylsphing-4-enine + UDP-alpha-D-glucose = a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + UDP + H(+)
2. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + H2O = beta-D-glucosyl-(1<->1)-sphing-4-enine + a fatty acid

Catalysed by UGCG, ASAH1

1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
2. (9Z)-hexadecenoyl-CoA + cholesterol = cholesteryl (9Z)-hexadecenoate + CoA

Catalysed by GBA1, GBA2, SOAT1

1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
2. (9Z,12Z,15Z)-octadecatrienoyl-CoA + cholesterol = (9Z,12Z,15Z-octadecatrienoyl)-cholesterol + CoA

Catalysed by GBA1, GBA2, SOAT2One of 2 routes

1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
2. 1-hexadecanoyl-2-(8Z,11Z,14Z-eicosatrienoyl)-sn-glycero-3-phosphocholine + cholesterol = cholesteryl (8Z,11Z,14Z)-eicosatrienoate + 1-hexadecanoyl-sn-glycero-3-phosphocholine

Catalysed by GBA1, GBA2, LCAT

1. acyl-CoA:sphingosine N-acyltransferase
2. acyl-CoA pool formation and breakdown

Catalysed by ASAH1, ACER2, ACER1, ASAH2Golgi apparatus, cytosol

1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
2. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + cholesterol = (5Z,8Z,11Z,14Z,17Z-eicosapentaenoyl)-cholesterol + CoA

Catalysed by GBA1, GBA2, SOAT2One of 2 routes

1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
2. cholesterol + reduced [NADPH--hemoprotein reductase] + O2 = 4beta-hydroxycholesterol + oxidized [NADPH--hemoprotein reductase] + H2O + H(+)

Catalysed by GBA1, GBA2, CYP3A4

1. acyl-CoA:sphingosine N-acyltransferase
2. an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)

Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 2 moreGolgi apparatus

1. acyl-CoA:sphingosine N-acyltransferase
2. acyl-CoA pool formation and breakdown

Catalysed by ASAH1, ACER2, ACER1, ASAH2Golgi apparatus, cytosol

1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
2. 1-hexadecanoyl-2-(5Z,8Z,11Z-eicosatrienoyl)-sn-glycero-3-phosphocholine + cholesterol = cholesteryl (5Z,8Z,11Z)-eicosatrienoate + 1-hexadecanoyl-sn-glycero-3-phosphocholine

Catalysed by GBA1, GBA2, LCAT

1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
2. 1-hexadecanoyl-2-(6Z,9Z,12Z-octadecatrienoyl)-sn-glycero-3-phosphocholine + cholesterol = (6Z,9Z,12Z-octadecatrienoyl)-cholesterol + 1-hexadecanoyl-sn-glycero-3-phosphocholine

Catalysed by GBA1, GBA2, LCAT

1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
2. (11Z)-octadecenoyl-CoA + cholesterol = cholesteryl (11Z)-octadecenoate + CoA

Catalysed by GBA1, GBA2, SOAT1

1. acyl-CoA:sphingosine N-acyltransferase
2. acyl-CoA pool formation and breakdown

Catalysed by ASAH1, ACER2, ACER1, ASAH2Golgi apparatus, cytosol

1. acyl-CoA:sphingosine N-acyltransferase
2. an acyl-CoA + acetyl-CoA = a 3-oxoacyl-CoA + CoA

Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 4 moreGolgi apparatus

1. acyl-CoA:sphingosine N-acyltransferase
2. acyl-CoA:sn-glycerol-3-phosphate 1-O-acyltransferase
3. Carnitine O-Palmitoyltransferase

Catalysed by ASAH1, ACER2, ACER1, ASAH2 and 7 moreGolgi apparatus, mitochondrial matrix, cytosolOne of 260 routes

1. a beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine + cholesterol = cholesteryl 3-beta-D-glucoside + an N-acylsphing-4-enine
2. 2 reduced [adrenodoxin] + cholesterol + O2 + 2 H(+) = (22R)-hydroxycholesterol + 2 oxidized [adrenodoxin] + H2O
3. (22R)-hydroxycholesterol + 2 reduced [adrenodoxin] + O2 + 2 H(+) = (20R,22R)-20,22-dihydroxycholesterol + 2 oxidized [adrenodoxin] + H2O