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).
This pathway has been updated with information from Lipidmaps>Sphingolipids [https://lipidmaps.org/resources/pathways/vanted.php]. Metabolites and proteins from this pathway are coloured orange and have a rounded rectangle shape (where an oval shape indicates that the node only occures in the LipidMaps pathway). Reactions occurring in the lipidmaps pathways are coloured orange (where a dashed line indicates that the reaction only occures in the LipidMaps pathway).c48c882.3.1.24(dihydro)ceramide synthasesWhere does this abbreviation stand for? dihydrosterigmatocystin?!2.3.1.50protein phosphatase 1 (PP1)protein-serine/threonine phosphataseCeramidase1.1.1.102C16 Dihydroceramide-1-PhosphateC16 DihydroLactosylceramide3-ketodihydrosphingosine reductase(dihydro)ceramide synthases. ASAH3 is old name of ACER1dihydrosphingomyelin(dihydro)ceramide synthases(dihydro)ceramide synthases. ASAH3 is old name of ACER1C16 Dihydro-Glucosyl- ceramidesphinganine-1-phosphatesphingosine-1-phosphate (S1P) lyaseC16 Dihydro-GalactoCerebroside4.1.2.272.7.8.3Glucosylceramide2.7.8.32.7.1.138protein phosphatase 2A (PP2A)2.3.1.24protein phosphatase 1 (PP1)Glucosylceramidaseprotein phosphatase 2A (PP2A)UGT8 / CGTglucosylceramide synthase (GCS)UGT8 / CGTglucosylceramide synthase (GCS)Sphingomyelin synthase 2Sphingomyelin synthase 1sphinganine-1-phosphatef3ef3esphingolipid metabolic pathwayPW:0000197Pathway Ontology19029065PubMedSphingolipidomics: a valuable tool for understanding the roles of sphingolipids in biology and disease.J Lipid Res2009Merrill AH JrStokes THMomin APark HPortz BJKelly SWang ESullards MCWang MD17928229PubMed(Glyco)sphingolipidology: an amazing challenge and opportunity for systems biology.Trends Biochem Sci2007Merrill AH JrWang MDPark MSullards MC18216770PubMedPrinciples of bioactive lipid signalling: lessons from sphingolipids.Nature reviews Molecular cell biology2008Hannun YAObeid LM