Category: membrane
Sphingoid-backbone lipids — sphingomyelin (membrane), ceramide (signaling + apoptosis induction), glucosylceramide (precursor for complex glycosphingolipids + gangliosides). De novo synthesis: serine + palmitoyl-CoA → 3-ketosphinganine (SPT) → sphinganine → ceramide (CerS). Sphingomyelin synthase + glucosylceramide synthase build the complex lipids. Lysosomal storage diseases of sphingolipid degradation: Gaucher (glucocerebrosidase), Niemann-Pick A/B (acid sphingomyelinase), Fabry (α-galactosidase A), Krabbe (galactocerebrosidase), Tay-Sachs (β-hexosaminidase A). Eliglustat / miglustat are oral glucosylceramide synthase inhibitors for Gaucher (substrate reduction therapy, vs ERT alternatives).