Sphingolipid pathway (Homo sapiens)
From WikiPathways
Description
The sphingolipid metabolites and genes (with the gene abbreviations shown in boxes, or enzyme names where gene names are ambiguous) are given for the condensation of serine and palmitoyl-CoA to form 3-ketosphinganine (3-ketoSa) by serine palmitoyltransferase, which is reduced to sphinganine (Sa), acylated to dihydroceramides, DHCer, by (DH)Cer synthases, and incorporated into more complex DH-sphingolipids (the 1-phosphate, DHCerP, sphingomyelins, DHSM, glucosylceramides, DHGlcCer, galactosylceramides, DHGalCer, lactosylceramides, DHLacCer, and sulfatides, or desaturated to Cer followed by headgroup addition. Also included are a number of the catabolic genes, e.g., sphingomyelinases, SMases, ceramidases, ASAH, sphingosine kinases, for the formation of sphinganine 1-phosphate (Sa1P) and sphingosine 1-phosphate (So1P), and phosphatases for the reverse reaction and the lyase that cleaves sphingoid base 1-phosphates to ethanolamine phosphate (EP), hexadecanal (C16:0al) and hexadecenal (C16:1al).
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Ontology Terms
Bibliography
- Merrill AH Jr, Stokes TH, Momin A, Park H, Portz BJ, Kelly S, Wang E, Sullards MC, Wang MD; ''Sphingolipidomics: a valuable tool for understanding the roles of sphingolipids in biology and disease.''; J Lipid Res, 2009 PubMed Europe PMC Scholia
- Hannun YA, Obeid LM; ''Principles of bioactive lipid signalling: lessons from sphingolipids.''; Nature reviews Molecular cell biology, 2008 PubMed Europe PMC Scholia
- Merrill AH Jr, Wang MD, Park M, Sullards MC; ''(Glyco)sphingolipidology: an amazing challenge and opportunity for systems biology.''; Trends Biochem Sci, 2007 PubMed Europe PMC Scholia
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External references
DataNodes
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Name | Type | Database reference | Comment |
---|---|---|---|
3-ketodihydroshpingosine | Metabolite | Q27109281 (Wikidata) | |
3-ketosphinganine | Metabolite | CHEBI:17862 (ChEBI) | |
ASAH1 | GeneProduct | 427 (Entrez Gene) | (dihydro)ceramide synthases |
ASAH2 | GeneProduct | 56624 (Entrez Gene) | |
ASAH3 | GeneProduct | (dihydro)ceramide synthases | |
B4GALT6 | GeneProduct | 9331 (Entrez Gene) | |
C16:0-al | Metabolite | ||
C16:1-al | Metabolite | ||
CDase | Protein | 3.5.1.23 (Enzyme Nomenclature) | Ceramidase |
CERK | GeneProduct | 64781 (Entrez Gene) | 2.7.1.138 |
CERT | GeneProduct | ||
Cer1P | Metabolite | CHEBI:13956 (ChEBI) | |
CerS1 | GeneProduct | 57515 (Entrez Gene) | 2.3.1.24 |
CerS2 | GeneProduct | 29956 (Entrez Gene) | |
CerS3 | GeneProduct | 204219 (Entrez Gene) | |
CerS4 | GeneProduct | 79603 (Entrez Gene) | |
CerS5 | GeneProduct | 91012 (Entrez Gene) | |
CerS6 | GeneProduct | 253782 (Entrez Gene) | |
Ceramides | Metabolite | HMDB04949 (HMDB) | |
DES1 | GeneProduct | ||
DES2 | GeneProduct | ||
DHCer1P | Metabolite | Q37611218 (Wikidata) | C16 Dihydroceramide-1-Phosphate |
DHGalCer | Metabolite | C16 Dihydro-GalactoCerebroside | |
DHGlcCer | Metabolite | LMSP0501AA04 (LIPID MAPS) | C16 Dihydro-Glucosyl- ceramide |
DHLacCer | Metabolite | LMSP0501AB14 (LIPID MAPS) | C16 DihydroLactosylceramide |
DHSM | Metabolite | LMSP030100A0 (LIPID MAPS) | dihydrosphingomyelin |
DHST | Metabolite | Where does this abbreviation stand for? dihydrosterigmatocystin?! | |
Dihydroceramide | Metabolite | HMDB06752 (HMDB) | |
Dihydrosphingosine | Metabolite | Q27088847 (Wikidata) | |
Ethanolamine phosphate | Metabolite | CHEBI:17553 (ChEBI) | |
FVT1 | GeneProduct | 2531 (Entrez Gene) | 1.1.1.102 |
Fumonisins B1 | Metabolite | Q420762 (Wikidata) | |
GAL3ST1 | GeneProduct | 9514 (Entrez Gene) | |
GBA2 | Protein | 57704 (Entrez Gene) | protein phosphatase 2A (PP2A) |
GBA | Protein | 2629 (Entrez Gene) | protein phosphatase 1 (PP1) |
GalCer Synthase | GeneProduct | ||
GalCer | Metabolite | CHEBI:36498 (ChEBI) | |
GlcCer Synthase | GeneProduct | GCase | |
GlcCer | Metabolite | HMDB00140 (HMDB) | Glucosylceramide |
Glucosylceramidase | Protein | 3.2.1.45 (Enzyme Nomenclature) | Glucosylceramidase |
KDSR | GeneProduct | ENSG00000119537 (Ensembl) | 3-ketodihydrosphingosine reductase |
Lactosylceramide | Metabolite | HMDB06750 (HMDB) | |
PP1 | Protein | 5499 (Entrez Gene) | protein phosphatase 1 (PP1) |
PP2A | Protein | 5515 (Entrez Gene) | protein phosphatase 2A (PP2A) |
PPAP2A | GeneProduct | 8611 (Entrez Gene) | |
Palmityl-CoA | Metabolite | HMDB01338 (HMDB) | |
Phosphatase | Protein | 3.1.3.16 (Enzyme Nomenclature) | protein-serine/threonine phosphatase |
S1P lyase | Protein | 4.1.2.27 (Enzyme Nomenclature) | sphingosine-1-phosphate (S1P) lyase |
SGPP2 | GeneProduct | 130367 (Entrez Gene) | |
SMS1 | Protein | Q86VZ5 (Uniprot-TrEMBL) | 2.7.8.3 |
SMS2 | Protein | Q8NHU3 (Uniprot-TrEMBL) | 2.7.8.3 |
SMase1 | GeneProduct | ||
SMase2 | GeneProduct | ||
SPHK1 | GeneProduct | 8877 (Entrez Gene) | |
SPHK2 | GeneProduct | 56848 (Entrez Gene) | |
SPT1 | GeneProduct | 10558 (Entrez Gene) | |
SPT2 | GeneProduct | 9517 (Entrez Gene) | 2.3.1.50 |
SPT3 | GeneProduct | 55304 (Entrez Gene) | |
Sa 1-phosphate | Metabolite | HMDB01383 (HMDB) | sphinganine-1-phosphate |
SaP lyase1 | GeneProduct | 4.1.2.27 | |
Serine | Metabolite | HMDB00187 (HMDB) | |
SoP lyase1 | GeneProduct | 4.1.2.27 (Enzyme Nomenclature) | 4.1.2.27 |
Sphinganine | Metabolite | HMDB00269 (undefined) | |
Sphingomyelin | Metabolite | HMDB01348 (HMDB) | |
Sphingosine 1-phosphate | Metabolite | HMDB00277 (HMDB) | |
Sphingosine | Metabolite | HMDB00252 (HMDB) | |
Sulfatide | Metabolite | HMDB00024 (HMDB) |
Annotated Interactions
No annotated interactions