Characterization of cholesterol homeostasis in sphingosine-1-phosphate lyase-deficient fibroblasts reveals a Niemann-Pick disease type C-like phenotype with enhanced lysosomal Ca(2+) storage

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...

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Bibliografische gegevens
Gepubliceerd in:Sci Rep
Hoofdauteurs: 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
Formaat: Artigo
Taal:Inglês
Gepubliceerd in: Nature Publishing Group 2017
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Article
Online toegang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5337937/
https://ncbi.nlm.nih.gov/pubmed/28262793
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/srep43575
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