Sphingolipid metabolism overview (Homo sapiens)

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Description

This pathway is inspired by the Lipidmaps>Sphingolipids pathway [[1](https://lipidmaps.org/pathway/pathways_maps)].

Sphingolipids are a class of lipids, which act as signal transduction and cell recognition molecules. This pathway provides an overview of the different tail lengths (on the right), which are used to convert sphinganine to DH-ceramide. All products are annotated with a lipid-class identifier.

The integrated pathway can be found [here](https://www.wikipathways.org/index.php/Pathway:WP4690).

Comments

HomologyMapper 
This pathway was inferred from Mus musculus pathway [WP4344_107206.gpml] with a 92.0% conversion rate.

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Quality Tags

Ontology Terms

 

Bibliography

  1. Han G, Gupta SD, Gable K, Niranjanakumari S, Moitra P, Eichler F, Brown RH Jr, Harmon JM, Dunn TM; ''Identification of small subunits of mammalian serinepalmitoyltransferase that confer distinct acyl-CoA substrate specificities.''; Proc Natl Acad Sci U S A, 2009 PubMed Europe PMC Scholia
  2. 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
  3. Merrill AH Jr; ''De novo sphingolipid biosynthesis: a necessary, but dangerous, pathway.''; J Biol Chem, 2002 PubMed Europe PMC Scholia
  4. 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; ''''; , PubMed Europe PMC Scholia

History

View all...
CompareRevisionActionTimeUserComment
139832view11:21, 7 July 2025DdiglesDeleted hidden old description
139653view05:52, 27 June 2025EgonwWater with unicode
137448view12:47, 2 March 2025EgonwModified description
128379view19:21, 3 February 2024Ash iyerFixed interactions to datanodes.
128378view19:20, 3 February 2024Ash iyerAdded grahical elements to datanodes.
128377view19:09, 3 February 2024Ash iyerAdded PPAP2A and PPAP2C identifiers
128289view02:04, 30 January 2024EweitzTailor node dimensions, soften disease color, standardize case
126692view13:12, 13 June 2023Conroy lipidsmore rhea/EC
126690view12:22, 13 June 2023Conroy lipidsadded Thea, EC, corrected wrong LMID, enzymes > Uniprot. 1st half
124751view08:56, 8 December 2022DeSlUpdated link to LIPID MAPS PWs.
117175view09:56, 18 May 2021EweitzModified title
115610view16:06, 1 March 2021DeSlLayout change
115609view16:05, 1 March 2021DeSlAdded Niemann pick disease B
107602view15:21, 23 October 2019DeSlNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
(2E)-hexadecenalMetaboliteLMFA06000089 (LIPID MAPS)
3-keto-sphinganineMetaboliteLMSP01020002 (LIPID MAPS)
ADPMetaboliteCHEBI:456216 (ChEBI)
ASAH1GeneProductQ13510 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = S:Q9WV54
ATPMetaboliteCHEBI:30616 (ChEBI)
CERKGeneProductQ8TCT0 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:223753
CERS1ProteinENSG00000223802 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = S:P27545
CERS2ProteinENSG00000143418 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:76893
CERS3ProteinENSG00000154227 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = S:Q1A3B0
CERS4ProteinENSG00000090661 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:67260
CERS6ProteinENSG00000172292 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:241447
Cer-1-PMetaboliteLMSP02050000 (LIPID MAPS)
CeramideMetaboliteLMSP02010000 (LIPID MAPS)
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)
CoA(26:1)Metabolite
DEGS1GeneProductO15121 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:13244
DEGS2GeneProductQ6QHC5 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:70059
DH-Cer-1-PMetaboliteLMSP020500A0 (LIPID MAPS)
DH-CeramideMetaboliteLMSP02020000 (LIPID MAPS)
DH-Gal-CerMetabolite
DH-Glc-CerMetabolite
DH-SMMetaboliteLMSP030100A0 (LIPID MAPS)
Ethanolamine-phosphateMetabolite1015 (PubChem-compound)
GBA1GeneProductP04062 (Uniprot-TrEMBL)
GBA2GeneProductQ9HCG7 (Uniprot-TrEMBL)
GBA3GeneProductQ9H227 (Uniprot-TrEMBL)
Gal-CerMetaboliteLMSP0501AC00 (LIPID MAPS)
Glc-CerMetaboliteLMSP0501AA00 (LIPID MAPS)
HexadecanalMetaboliteLMFA06000088 (LIPID MAPS)
H₂OMetaboliteCHEBI:15377 (ChEBI)
KDSRProteinQ06136 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = S:Q6GV12
PLPP3GeneProductO14495 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:67916
Palmitoyl-CoAMetaboliteLMFA07050360 (LIPID MAPS) aka CoA(16:0)
Ppap2aProtein8611 (Entrez Gene) No homologues found for original id L:19012 Also, known as PLPP1
Ppap2cProtein8612 (Entrez Gene) Also known as PLPP2
SGMS1GeneProductQ86VZ5 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:208449
SGMS2ProteinQ8NHU3 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:74442
SGPL1ProteinENSG00000166224 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:20397
SGPP1GeneProductQ9BX95 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = S:Q9JI99
SGPP2GeneProductQ8IWX5 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = S:Q810K3
SMMetaboliteLMSP0301 (LIPID MAPS)
SMPD1ProteinP17405 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:20597
SPHK1GeneProductQ9NYA1 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = S:Q8CI15
SPHK2GeneProductQ9NRA0 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = S:Q9JIA7
SPTLC1GeneProductO15269 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:268656
SPTLC2GeneProductO15270 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = S:P97363
SerineMetabolite5951 (PubChem-compound)
Sphinganine-1-phosphateMetaboliteLMSP01050002 (LIPID MAPS)
SphinganineMetaboliteLMSP01020001 (LIPID MAPS)
Sphingosine-1-phosphateMetaboliteLMSP01050001 (LIPID MAPS)
SphingosineMetaboliteLMSP01010001 (LIPID MAPS)
UGCGProteinQ16739 (Uniprot-TrEMBL) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:22234
UGT8ProteinENSG00000174607 (Ensembl) EC:2.4.1.47

Annotated Interactions

View all...
SourceTargetTypeDatabase referenceComment
3-keto-sphinganineSphinganinemim-conversion22642 (Rhea) EC:1.1.1.102
ADPmim-conversion15466 (Rhea)
ATPmim-conversion15466 (Rhea)
CeramideCer-1-Pmim-conversion17930 (Rhea) EC:2.7.1.138
CeramideGal-Cermim-conversion13094 (Rhea) EC:2.4.1.47
CeramideGlc-Cermim-conversion12089 (Rhea) EC:2.4.1.80
CeramideSMmim-conversion18766 (Rhea) EC2.7.8.27
CeramideSphingosinemim-conversion20857 (Rhea) EC:3.5.1.23
DH-CeramideCeramidemim-conversion46545 (Rhea) EC:1.14.19.17
DH-CeramideDH-SMmim-conversion44621 (Rhea)
Glc-CerCeramidemim-conversion13270 (Rhea) EC:3.2.1.45
H₂Omim-conversion27515 (Rhea)
Palmitoyl-CoA3-keto-sphinganinemim-conversion14762 (Rhea) EC:2.3.1.50
SMCeramidemim-conversion19254 (Rhea) 3.1.4.12
Sphinganine-1-phosphateHexadecanalmim-conversion18594 (Rhea) EC:4.1.2.27
Sphinganine-1-phosphateSphinganinemim-conversion27515 (Rhea)
SphinganineSphinganine-1-phosphatemim-conversion15466 (Rhea) EC:2.7.1.91
Sphinganinemim-conversion34736 (Rhea) EC:2.3.1.24
Sphingosine-1-phosphate(2E)-hexadecenalmim-conversion33508 (Rhea) EC:4.1.2.27
Sphingosine-1-phosphateSphingosinemim-conversion27519 (Rhea)
SphingosineSphingosine-1-phosphatemim-conversion35848 (Rhea) EC:2.7.1.91
UGT8mim-catalysis13094 (Rhea)
Retrieved from "https://classic.wikipathways.org/index.php/Pathway:WP4725"
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