Sphingolipid metabolism (integrated pathway) (WP4690)

Mus musculus

Overview PW


Denise Slenter , Eric Weitz , and Conroy lipids


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Mus musculus


Lipids and LIPID MAPS


Pathway Ontology

sphingolipid biosynthetic pathway sphingolipid metabolic pathway sphingomyelin metabolic pathway


Label Type Compact URI Comment
ATP Metabolite chebi:30616
3-keto-sphinganine Metabolite lipidmaps:LMSP01020002
Palmitoyl-CoA Metabolite lipidmaps:LMFA07050360 aka CoA(16:0)
Serine Metabolite pubchem.compound:5951
Hexadecanal Metabolite lipidmaps:LMFA06000088
Ethanolamine-phosphate Metabolite pubchem.compound:1015
H2O Metabolite chebi:15377
Sphingosine Metabolite lipidmaps:LMSP01010001
Sphinganine Metabolite lipidmaps:LMSP01020001
Sphinganine-1-phosphate Metabolite lipidmaps:LMSP01050002
C16 SM Metabolite lipidmaps:LMSP03010003 SM = sphingomyelin
C16 GlcCer Metabolite lipidmaps:LMSP0501AA03
C16DH SM Metabolite lipidmaps:LMSP03010004 SM = sphingomyelin
C16DH GlcCer Metabolite lipidmaps:LMSP0501AA04
Sphingosine-1-phosphate Metabolite lipidmaps:LMSP01050001
1-Hexadecenal Metabolite lipidmaps:LMFA06000088
CoA(16:0) Metabolite lipidmaps:LMFA07050360
CoA(18:0) Metabolite lipidmaps:LMFA07050369
CoA(20:0) Metabolite lipidmaps:LMFA07050329
CoA(22:0) Metabolite lipidmaps:LMFA07050289 AKA 'Coenzyme A, S-​docosanoate'
CoA(24:0) Metabolite lipidmaps:LMFA07050372 AKA 'Coenzyme A, S-​(tetracosanoate)​'
CoA(24:1) Metabolite lipidmaps:LMFA07050098 AKA 'Coenzyme A, S-​(15Z)​-​15-​tetracosenoate'
C16DH-Cer Metabolite lipidmaps:LMSP02020001
CoA(26:0) Metabolite lipidmaps:LMFA07050327 AKA 'Coenzyme A, S-​hexacosanoate'
CoA(26:1) Metabolite lipidmaps:LMFA07050099
ADP Metabolite chebi:456216
C16 Cer Metabolite lipidmaps:LMSP02010004
C18 SM Metabolite lipidmaps:LMSP03010001 SM = sphingomyelin
C18 GlcCer Metabolite lipidmaps:LMSP0501AA05
C18DH SM Metabolite lipidmaps:LMSP03010020 SM = sphingomyelin
C18DH GlcCer Metabolite lipidmaps:LMSP0501AA19
C18DH-Cer Metabolite lipidmaps:LMSP02020008
C18 Cer Metabolite lipidmaps:LMSP02010006
C20 SM Metabolite lipidmaps:LMSP03010005 SM = sphingomyelin
C20 GlcCer Metabolite lipidmaps:LMSP0501AA06
C20DH SM Metabolite lipidmaps:LMSP03010021 SM = sphingomyelin
C20DH GlcCer Metabolite lipidmaps:LMSP0501AA20
C20DH-Cer Metabolite lipidmaps:LMSP02020009
C20 Cer Metabolite lipidmaps:LMSP02010007
C22 SM Metabolite lipidmaps:LMSP03010006 SM = sphingomyelin
C22 GlcCer Metabolite lipidmaps:LMSP0501AA07
C22DH SM Metabolite lipidmaps:LMSP03010022 SM = sphingomyelin
C22DH GlcCer Metabolite lipidmaps:LMSP0501AA21
C22DH-Cer Metabolite lipidmaps:LMSP02020010
C22 Cer Metabolite lipidmaps:LMSP02010008
C24 SM Metabolite lipidmaps:LMSP03010008 SM = sphingomyelin
C24 GlcCer Metabolite lipidmaps:LMSP0501AA09
C24DH SM Metabolite lipidmaps:LMSP03010024 SM = sphingomyelin
C24DH GlcCer Metabolite lipidmaps:LMSP0501AA23
C24DH-Cer Metabolite lipidmaps:LMSP02020012
C24 Cer Metabolite lipidmaps:LMSP02010012
C24:1 SM Metabolite lipidmaps:LMSP03010007 SM = sphingomyelin
C24:1 GlcCer Metabolite lipidmaps:LMSP0501AA08
C24:1DH SM Metabolite lipidmaps:LMSP03010023 SM = sphingomyelin
C24:1DH GlcCer Metabolite lipidmaps:LMSP0501AA22
C24:1DH-Cer Metabolite lipidmaps:LMSP02020011
C24:1 Cer Metabolite lipidmaps:LMSP02010009
C26:1 SM Metabolite lipidmaps:LMSP03010009 SM = sphingomyelin
C26:1 GlcCer Metabolite lipidmaps:LMSP0501AA10
C26:1DH SM Metabolite lipidmaps:LMSP03010025 SM = sphingomyelin
C26:1DH GlcCer Metabolite lipidmaps:LMSP0501AA24
C26:1DH-Cer Metabolite lipidmaps:LMSP02020013
C26:1 Cer Metabolite lipidmaps:LMSP02010010
C26 SM Metabolite lipidmaps:LMSP03010010 SM = sphingomyelin
C26 GlcCer Metabolite lipidmaps:LMSP0501AA11
C26DH SM Metabolite lipidmaps:LMSP03010026 SM = sphingomyelin
C26DH GlcCer Metabolite lipidmaps:LMSP0501AA25
C26DH-Cer Metabolite lipidmaps:LMSP02020014
C26 Cer Metabolite lipidmaps:LMSP02010011
Ethanolamine-phosphate Metabolite pubchem.compound:1015
Ppap2a Protein ncbigene:19012
Cerk Protein ncbigene:223753
Sgpp1 Protein uniprot:Q9JI99
Asah1 Protein uniprot:Q9WV54
Sgms1 Protein ncbigene:208449
Sphk2 Protein uniprot:Q9JIA7
Sgpl1 Protein ncbigene:20397
Ugcg Protein ncbigene:22234
Sptlc1 Protein ncbigene:268656
Sphk1 Protein uniprot:Q8CI15
Degs1 Protein ncbigene:13244
Kdsr Protein uniprot:Q6GV12 AKA Fvt1
Sptlc2 Protein uniprot:P97363
CerS4 Protein ncbigene:67260
CerS5 Protein uniprot:Q9D6K9 AKA Lass5
CerS6 Protein uniprot:Q8C172 AKA Lass6
Smpd1 Protein ncbigene:20597
Degs2 Protein ncbigene:70059
Sgms2 Protein ncbigene:74442
Ppap2b Protein ncbigene:67916
Ppap2c Protein ncbiprotein:50784
Ugt8a Protein ncbigene:22239
CerS1 Protein uniprot:P27545
CerS4 Protein ncbigene:67260
Cers2 Protein ncbigene:76893 Original LipidMaps annotation
Cers3 Protein uniprot:Q1A3B0
Sgpp2 Protein uniprot:Q810K3
Sphk2 Protein uniprot:Q9JIA7
Sphk1 Protein uniprot:Q8CI15
Sgpl1 Protein ncbigene:20397
CerS4 Protein ncbigene:67260
Cers2 Protein ncbigene:76893
CerS4 Protein ncbigene:67260 Protein found in literature (not in original LipidMapsPW)
CerS5 Protein uniprot:Q9D6K9 AKA Lass5
CerS6 Protein uniprot:Q8C172 AKA Lass6
Cerk Protein ncbigene:223753
Ugcg Protein ncbigene:22234
Smpd1 Protein ncbigene:20597
Sgms1 Protein ncbigene:208449
Sgms2 Protein ncbigene:74442
Ugt8a Protein ncbigene:22239
Cers3 Protein uniprot:Q1A3B0


  1. Characterization of murine sphingosine-1-phosphate phosphohydrolase. Le Stunff H, Peterson C, Thornton R, Milstien S, Mandala SM, Spiegel S. J Biol Chem. 2002 Mar 15;277(11):8920–7. PubMed Europe PMC Scholia
  2. De novo sphingolipid biosynthesis: a necessary, but dangerous, pathway. Merrill AH Jr. J Biol Chem. 2002 Jul 19;277(29):25843–6. PubMed Europe PMC Scholia
  3. Mammalian Lass6 and its related family members regulate synthesis of specific ceramides. Mizutani Y, Kihara A, Igarashi Y. Biochem J. 2005 Aug 15;390(Pt 1):263–71. PubMed Europe PMC Scholia
  4. LASS5 is a bona fide dihydroceramide synthase that selectively utilizes palmitoyl-CoA as acyl donor. Lahiri S, Futerman AH. J Biol Chem. 2005 Oct 7;280(40):33735–8. PubMed Europe PMC Scholia
  5. Kinetic characterization of mammalian ceramide synthases: determination of K(m) values towards sphinganine. Lahiri S, Lee H, Mesicek J, Fuks Z, Haimovitz-Friedman A, Kolesnick RN, et al. FEBS Lett. 2007 Nov 13;581(27):5289–94. PubMed Europe PMC Scholia
  6. A mouse macrophage lipidome. Dennis EA, Deems RA, Harkewicz R, Quehenberger O, Brown HA, Milne SB, et al. J Biol Chem. 2010 Dec 17;285(51):39976–85. PubMed Europe PMC Scholia
  7. Ceramide Synthase 5 Is Essential to Maintain C16:0-Ceramide Pools and Contributes to the Development of Diet-induced Obesity. Gosejacob D, Jäger PS, Vom Dorp K, Frejno M, Carstensen AC, Köhnke M, et al. J Biol Chem. 2016 Mar 25;291(13):6989–7003. PubMed Europe PMC Scholia