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Differential Effects of 2-Hydroxypropyl-Cyclodextrins on Lipid Accumulation in Npc1-Null Cells

Niemann–Pick disease type C (NPC) is an autosomal recessive disorder characterized by abnormal accumulation of free cholesterol and sphingolipids in lysosomes. The iminosugar miglustat, which inhibits hexosylceramide synthesis, is used for NPC treatment, and 2-hydroxypropyl-β-cyclodextrin (HP-β-CD),...

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Detalles Bibliográficos
Autores principales: Hoque, Sanzana, Kondo, Yuki, Sakata, Nodoka, Yamada, Yusei, Fukaura, Madoka, Higashi, Taishi, Motoyama, Keiichi, Arima, Hidetoshi, Higaki, Katsumi, Hayashi, Akio, Komiya, Takaki, Ishitsuka, Yoichi, Irie, Tetsumi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7038050/
https://www.ncbi.nlm.nih.gov/pubmed/32019132
http://dx.doi.org/10.3390/ijms21030898