Sphingolipid metabolism (integrated pathway) (Mus musculus)

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7641High fat dietATP3-keto-sphinganinePpap2a3CerkSgpp1Asah1Palmitoyl-CoA3Sgms1Sphk2Sgpl1UgcgSerine3HexadecanalSptlc1Sphk1Degs1KdsrSptlc2Ethanolamine-phosphateH2OSphingosineSphinganineSphinganine-1-phosphate3CerS4CerS51, 2, 5CerS65C16 CerPC16DH CerPC16 SMC16 GlcCerC16DH SMC16DH GlcCerC16 GalCerC16DH GalCerSmpd1Degs2Sgms2Sphingosine-1-phosphate1-HexadecenalPpap2bPpap2cUgt8aCoA(16:0)CoA(18:0)CoA(20:0)CoA(22:0)CoA(24:0)CoA(24:1)C16DH-CerCerS15CerS4Cers25CoA(26:0)CoA(26:1)Cers3ADPSgpp2Sphk2Sphk1Sgpl1CerS4Cers25CerS45CoA(14:0)C14DH-CeramideCerS5CerS655C16 Cer3CerkUgcgSmpd1Sgms1Sgms2Ugt8aC18 CerPC18DH CerPC18 SMC18 GlcCerC18DH SMC18DH GlcCerC18 GalCerC18DH GalCerC18DH-CerC18 CerC20 CerPC20DH CerPC20 SMC20 GlcCerC20DH SMC20DH GlcCerC20 GalCerC20DH GalCerC20DH-CerC20 CerC22 CerPC22DH CerPC22 SMC22 GlcCerC22DH SMC22DH GlcCerC22 GalCerC22DH GalCerC22DH-CerC22 CerC24 CerPC24DH CerPC24 SMC24 GlcCerC24DH SMC24DH GlcCerC24 GalCerC24DH GalCerC24DH-CerC24 CerC24:1 CerPC24:1DH CerPC24:1 SMC24:1 GlcCerC24:1DH SMC24:1DH GlcCerC24:1 GalCerC24:1DH GalCerC24:1DH-CerC24:1 CerCers3C26:1 CerPC26:1DH CerPC26:1 SMC26:1 GlcCerC26:1DH SMC26:1DH GlcCerC26:1 GalCerC26:1DH GalCerC26:1DH-CerC26:1 CerC26 CerPC26DH CerPC26 SMC26 GlcCerC26DH SMC26DH GlcCerC26 GalCerC26DH GalCerC26DH-CerC26 CerEthanolamine-phosphate


This pathway is inspired by the Lipidmaps>Sphingolipids pathway (all chain lengths) [1].

Sphingolipids are a class of lipids, which act as signal transduction and cell recognition molecules. This pathway shows all different tail lengths and their conversions into specific products. All products are annotated with lipid-specific identifiers (if available).

The general overview pathway can be found here.

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Ontology Terms



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  1. Gosejacob D, Jäger PS, Vom Dorp K, Frejno M, Carstensen AC, Köhnke M, Degen J, Dörmann P, Hoch M; ''Ceramide Synthase 5 Is Essential to Maintain C16:0-Ceramide Pools and Contributes to the Development of Diet-induced Obesity.''; J Biol Chem, 2016 PubMed Europe PMC Scholia
  2. Lahiri S, Futerman AH; ''LASS5 is a bona fide dihydroceramide synthase that selectively utilizes palmitoyl-CoA as acyl donor.''; J Biol Chem, 2005 PubMed Europe PMC Scholia
  3. Merrill AH Jr; ''De novo sphingolipid biosynthesis: a necessary, but dangerous, pathway.''; J Biol Chem, 2002 PubMed Europe PMC Scholia
  4. Le Stunff H, Peterson C, Thornton R, Milstien S, Mandala SM, Spiegel S; ''Characterization of murine sphingosine-1-phosphate phosphohydrolase.''; J Biol Chem, 2002 PubMed Europe PMC Scholia
  5. Mizutani Y, Kihara A, Igarashi Y; ''Mammalian Lass6 and its related family members regulate synthesis of specific ceramides.''; Biochem J, 2005 PubMed Europe PMC Scholia
  6. Lahiri S, Lee H, Mesicek J, Fuks Z, Haimovitz-Friedman A, Kolesnick RN, Futerman AH; ''Kinetic characterization of mammalian ceramide synthases: determination of K(m) values towards sphinganine.''; FEBS Lett, 2007 PubMed Europe PMC Scholia
  7. Dennis EA, Deems RA, Harkewicz R, Quehenberger O, Brown HA, Milne SB, Myers DS, Glass CK, Hardiman G, Reichart D, Merrill AH Jr, Sullards MC, Wang E, Murphy RC, Raetz CR, Garrett TA, Guan Z, Ryan AC, Russell DW, McDonald JG, Thompson BM, Shaw WA, Sud M, Zhao Y, Gupta S, Maurya MR, Fahy E, Subramaniam S; ''A mouse macrophage lipidome.''; J Biol Chem, 2010 PubMed Europe PMC Scholia


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117946view10:46, 23 May 2021EweitzModified title
107209view14:30, 17 September 2019MaintBotChEBI identifier normalization
106335view08:54, 21 August 2019DeSlAdded general LipidMaps publication.
106334view08:53, 21 August 2019DeSlModified description
106269view14:17, 19 August 2019DeSlModified description
106268view14:17, 19 August 2019DeSlAnnotated specific lipids if part of tabular Data file of LipidMaps.
106267view14:05, 19 August 2019DeSlCompleted bottom part of PW.
106266view14:00, 19 August 2019DeSlAdd C26 length products.
106265view13:52, 19 August 2019DeSlAdded C22-C24 specific metabolite nodes.
106263view13:33, 19 August 2019DeSlOntology Term : 'sphingolipid biosynthetic pathway' added !
106262view13:32, 19 August 2019DeSlOntology Term : 'sphingomyelin metabolic pathway' added !
106255view13:27, 19 August 2019DeSlModified description
106252view13:25, 19 August 2019DeSlUpdated title
106251view13:24, 19 August 2019DeSlAdded specific metabolites for several chain lengths.
106249view12:55, 19 August 2019DeSlNew pathway

External references


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NameTypeDatabase referenceComment
1-HexadecenalMetaboliteLMFA06000088 (LIPID MAPS)
3-keto-sphinganineMetaboliteLMSP01020002 (LIPID MAPS)
ADPMetaboliteCHEBI:456216 (ChEBI)
ATPMetaboliteCHEBI:30616 (ChEBI)
Asah1ProteinQ9WV54 (Uniprot-TrEMBL)
C16 CerMetaboliteLMSP02010004 (LIPID MAPS)
C16 CerPMetabolite
C16 GalCerMetabolite
C16 GlcCerMetaboliteLMSP0501AA03 (LIPID MAPS)
C16 SMMetaboliteLMSP03010003 (LIPID MAPS) SM = sphingomyelin
C16DH CerPMetabolite
C16DH GalCerMetabolite
C16DH GlcCerMetaboliteLMSP0501AA04 (LIPID MAPS)
C16DH SMMetaboliteLMSP03010004 (LIPID MAPS) SM = sphingomyelin
C16DH-CerMetaboliteLMSP02020001 (LIPID MAPS)
C18 CerMetaboliteLMSP02010006 (LIPID MAPS)
C18 CerPMetabolite
C18 GalCerMetabolite
C18 GlcCerMetaboliteLMSP0501AA05 (LIPID MAPS)
C18 SMMetaboliteLMSP03010001 (LIPID MAPS) SM = sphingomyelin
C18DH CerPMetabolite
C18DH GalCerMetabolite
C18DH GlcCerMetaboliteLMSP0501AA19 (LIPID MAPS)
C18DH SMMetaboliteLMSP03010020 (LIPID MAPS) SM = sphingomyelin
C18DH-CerMetaboliteLMSP02020008 (LIPID MAPS)
C20 CerMetaboliteLMSP02010007 (LIPID MAPS)
C20 CerPMetabolite
C20 GalCerMetabolite
C20 GlcCerMetaboliteLMSP0501AA06 (LIPID MAPS)
C20 SMMetaboliteLMSP03010005 (LIPID MAPS) SM = sphingomyelin
C20DH CerPMetabolite
C20DH GalCerMetabolite
C20DH GlcCerMetaboliteLMSP0501AA20 (LIPID MAPS)
C20DH SMMetaboliteLMSP03010021 (LIPID MAPS) SM = sphingomyelin
C20DH-CerMetaboliteLMSP02020009 (LIPID MAPS)
C22 CerMetaboliteLMSP02010008 (LIPID MAPS)
C22 CerPMetabolite
C22 GalCerMetabolite
C22 GlcCerMetaboliteLMSP0501AA07 (LIPID MAPS)
C22 SMMetaboliteLMSP03010006 (LIPID MAPS) SM = sphingomyelin
C22DH CerPMetabolite
C22DH GalCerMetabolite
C22DH GlcCerMetaboliteLMSP0501AA21 (LIPID MAPS)
C22DH SMMetaboliteLMSP03010022 (LIPID MAPS) SM = sphingomyelin
C22DH-CerMetaboliteLMSP02020010 (LIPID MAPS)
C24 CerMetaboliteLMSP02010012 (LIPID MAPS)
C24 CerPMetabolite
C24 GalCerMetabolite
C24 GlcCerMetaboliteLMSP0501AA09 (LIPID MAPS)
C24 SMMetaboliteLMSP03010008 (LIPID MAPS) SM = sphingomyelin
C24:1 CerMetaboliteLMSP02010009 (LIPID MAPS)
C24:1 CerPMetabolite
C24:1 GalCerMetabolite
C24:1 GlcCerMetaboliteLMSP0501AA08 (LIPID MAPS)
C24:1 SMMetaboliteLMSP03010007 (LIPID MAPS) SM = sphingomyelin
C24:1DH CerPMetabolite
C24:1DH GalCerMetabolite
C24:1DH GlcCerMetaboliteLMSP0501AA22 (LIPID MAPS)
C24:1DH SMMetaboliteLMSP03010023 (LIPID MAPS) SM = sphingomyelin
C24:1DH-CerMetaboliteLMSP02020011 (LIPID MAPS)
C24DH CerPMetabolite
C24DH GalCerMetabolite
C24DH GlcCerMetaboliteLMSP0501AA23 (LIPID MAPS)
C24DH SMMetaboliteLMSP03010024 (LIPID MAPS) SM = sphingomyelin
C24DH-CerMetaboliteLMSP02020012 (LIPID MAPS)
C26 CerMetaboliteLMSP02010011 (LIPID MAPS)
C26 CerPMetabolite
C26 GalCerMetabolite
C26 GlcCerMetaboliteLMSP0501AA11 (LIPID MAPS)
C26 SMMetaboliteLMSP03010010 (LIPID MAPS) SM = sphingomyelin
C26:1 CerMetaboliteLMSP02010010 (LIPID MAPS)
C26:1 CerPMetabolite
C26:1 GalCerMetabolite
C26:1 GlcCerMetaboliteLMSP0501AA10 (LIPID MAPS)
C26:1 SMMetaboliteLMSP03010009 (LIPID MAPS) SM = sphingomyelin
C26:1DH CerPMetabolite
C26:1DH GalCerMetabolite
C26:1DH GlcCerMetaboliteLMSP0501AA24 (LIPID MAPS)
C26:1DH SMMetaboliteLMSP03010025 (LIPID MAPS) SM = sphingomyelin
C26:1DH-CerMetaboliteLMSP02020013 (LIPID MAPS)
C26DH CerPMetabolite
C26DH GalCerMetabolite
C26DH GlcCerMetaboliteLMSP0501AA25 (LIPID MAPS)
C26DH SMMetaboliteLMSP03010026 (LIPID MAPS) SM = sphingomyelin
C26DH-CerMetaboliteLMSP02020014 (LIPID MAPS)
CerS1ProteinP27545 (Uniprot-TrEMBL)
CerS4Protein67260 (Entrez Gene) Protein found in literature (not in original LipidMapsPW)
CerS5ProteinQ9D6K9 (Uniprot-TrEMBL) AKA Lass5
CerS6ProteinQ8C172 (Uniprot-TrEMBL) AKA Lass6
CerkProtein223753 (Entrez Gene)
Cers2Protein76893 (Entrez Gene)
Cers3ProteinQ1A3B0 (Uniprot-TrEMBL)
CoA(16:0)MetaboliteLMFA07050360 (LIPID MAPS)
CoA(18:0)MetaboliteCHEBI:15541 (ChEBI)
CoA(20:0)MetaboliteCHEBI:15527 (ChEBI)
CoA(22:0)Metabolite24330-89-0 (CAS) AKA "Coenzyme A, S-​docosanoate"
CoA(24:0)Metabolite24305-30-4 (CAS) AKA "Coenzyme A, S-​(tetracosanoate)​"
CoA(24:1)Metabolite88362-35-0 (CAS) AKA "Coenzyme A, S-​(15Z)​-​15-​tetracosenoate"
CoA(26:0)Metabolite99313-57-2 (CAS) AKA "Coenzyme A, S-​hexacosanoate"
Degs1Protein13244 (Entrez Gene)
Degs2Protein70059 (Entrez Gene)
Ethanolamine-phosphateMetabolite1015 (PubChem-compound)
H2OMetaboliteCHEBI:15377 (ChEBI)
HexadecanalMetaboliteLMFA06000088 (LIPID MAPS)
KdsrProteinQ6GV12 (Uniprot-TrEMBL) AKA Fvt1
Palmitoyl-CoAMetaboliteLMFA07050360 (LIPID MAPS) aka CoA(16:0)
Ppap2aProtein19012 (Entrez Gene)
Ppap2bProtein67916 (Entrez Gene)
Ppap2cProtein50784 (NCBI Protein)
SerineMetabolite5951 (PubChem-compound)
Sgms1Protein208449 (Entrez Gene)
Sgms2Protein74442 (Entrez Gene)
Sgpl1Protein20397 (Entrez Gene)
Sgpp1ProteinQ9JI99 (Uniprot-TrEMBL)
Sgpp2ProteinQ810K3 (Uniprot-TrEMBL)
Smpd1Protein20597 (Entrez Gene)
Sphinganine-1-phosphateMetaboliteLMSP01050002 (LIPID MAPS)
SphinganineMetaboliteLMSP01020001 (LIPID MAPS)
Sphingosine-1-phosphateMetaboliteLMSP01050001 (LIPID MAPS)
SphingosineMetaboliteLMSP01010001 (LIPID MAPS)
Sphk1ProteinQ8CI15 (Uniprot-TrEMBL)
Sphk2ProteinQ9JIA7 (Uniprot-TrEMBL)
Sptlc1Protein268656 (Entrez Gene)
Sptlc2ProteinP97363 (Uniprot-TrEMBL)
UgcgProtein22234 (Entrez Gene)
Ugt8aProtein22239 (Entrez Gene)

Annotated Interactions

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SourceTargetTypeDatabase referenceComment
3-keto-sphinganineSphinganinemim-conversion22642 (Rhea)
ADPmim-conversion15466 (Rhea)
ATPmim-conversion15466 (Rhea)
CoA(16:0)mim-conversion36540 (Rhea)
H2Omim-conversion27515 (Rhea)
Palmitoyl-CoA3-keto-sphinganinemim-conversion14762 (Rhea)
Sphinganine-1-phosphateSphinganinemim-conversion27515 (Rhea)
SphinganineSphinganine-1-phosphatemim-conversion15466 (Rhea)
Sphinganinemim-conversion36540 (Rhea) Kinetic info: [PMID:17977534]
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