Article
Characterization of cholesterol homeostasis in sphingosine-1-phosphate lyase-deficient fibroblasts reveals a Niemann-Pick disease type C-like phenotype with enhanced lysosomal Ca2+ storage.
Scientific reports - 6 Mar 2017
Vienken Hans, Mabrouki Nathalie, Grabau Katja, Claas Ralf Frederik, Rudowski Agnes, Schömel Nina, Pfeilschifter Josef, Lütjohann Dieter, van Echten-Deckert Gerhild, Meyer Zu Heringdorf Dagmar
Abstract excerpt
Sphingosine-1-phosphate (S1P) lyase irreversibly cleaves S1P, thereby catalysing the ultimate step of sphingolipid degradation. We show here that embryonic fibroblasts from S1P lyase-deficient mice (Sgpl1-/--MEFs), in which S1P and sphingosine accumulate, have features of Niemann-Pick disease type C (NPC) cells. In the presence of serum, overall cholesterol content was elevated in Sgpl1-/--MEFs, due to...
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