De Novo Sphingolipid Biosynthesis: A Necessary, but Dangerous, Pathway*

By Alfred H. Merrill Jr.. Journal of Biological Chemistry, 2002

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Differential expression of (dihydro)ceramide synthases in mouse brain: oligodendrocyte-specific expression of CerS2/Lass2 (2008, neutral): “In addition, CerS can acylate sphingosine generated by degradation of sphingolipids (Merrill 2002).”
Serine palmitoyltransferase, a key enzyme of sphingolipid metabolism. (2003, background): “In mammalian cells, dihydrosphingosine is N-acylated to form dihydroceramide, which is then desaturated to form ceramide [15].”
Lip1p: a novel subunit of acyl‐CoA ceramide synthase (2005, background): “Moreover, the balance between sphingoid bases (DHS/PHS/sphingosine), their phosphorylated counterparts and ceramide seems to control cell fate (Merrill, 2002; Spiegel and Milstien, 2002).”
Discovery of the molecular machinery CERT for endoplasmic reticulum-to-Golgi trafficking of ceramide (2006, background): “The main pathway of de novo synthesis of sphingolipids is shown [46].”
Fumonisins disrupt sphingolipid metabolism, folate transport, and neural tube development in embryo culture and in vivo: a potential risk factor for human neural tube defects among populations consuming fumonisin-contaminated maize. (2004, background): “Fumonisins inhibit ceramide synthase because they have structural features that resemble the cosubstrates (sphingoid bases and fatty acyl-CoA’s) (14,22), and inhibition has been characterized in vitro as well as in numerous cell lines, animals, plants, and fungi (14,15).”