Normalization of Hepatic Homeostasis in the Npc1(nmf164) Mouse Model of Niemann-Pick Type C Disease Treated with the Histone Deacetylase Inhibitor Vorinostat

Niemann-Pick type C (NP-C) disease is a fatal genetic lipidosis for which there is no Food and Drug Administration (FDA)-approved therapy. Vorinostat, an FDA-approved inhibitor of histone deacetylases, ameliorates lysosomal lipid accumulation in cultured NP-C patient fibroblasts. To assess the thera...

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Enregistré dans:
Détails bibliographiques
Publié dans:J Biol Chem
Auteurs principaux: Munkacsi, Andrew B., Hammond, Natalie, Schneider, Remy T., Senanayake, Dinindu S., Higaki, Katsumi, Lagutin, Kirill, Bloor, Stephen J., Ory, Daniel S., Maue, Robert A., Chen, Fannie W., Hernandez-Ono, Antonio, Dahlson, Nicole, Repa, Joyce J., Ginsberg, Henry N., Ioannou, Yiannis A., Sturley, Stephen L.
Format: Artigo
Langue:Inglês
Publié: American Society for Biochemistry and Molecular Biology 2017
Sujets:
Lipids
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC5377760/
https://ncbi.nlm.nih.gov/pubmed/28031458
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M116.770578
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