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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...
Enregistré dans:
| Publié dans: | Sci Rep |
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| Auteurs principaux: | , , , , , , , , , |
| Format: | Artigo |
| Langue: | Inglês |
| Publié: |
Nature Publishing Group
2017
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| Sujets: | |
| Accès en ligne: | 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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