Normalization of Cholesterol Homeostasis by 2-Hydroxypropyl-β-cyclodextrin in Neurons and Glia from Niemann-Pick C1 (NPC1)-deficient Mice

Niemann-Pick C (NPC) disease is an inherited, progressive neurodegenerative disorder caused by mutations in the NPC1 or NPC2 gene that result in an accumulation of unesterified cholesterol in late endosomes/lysosomes (LE/L) and impaired export of cholesterol from LE/L to the endoplasmic reticulum (E...

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Veröffentlicht in:	The Journal of biological chemistry 2012-03, Vol.287 (12), p.9290-9298
Hauptverfasser:	Peake, Kyle B., Vance, Jean E.
Format:	Artikel
Sprache:	eng
Schlagworte:	2-Hydroxypropyl-beta-cyclodextrin Animals Astrocytes beta-Cyclodextrins - pharmacology Cholesterol - metabolism Cholesterol Regulation Endosomes - drug effects Endosomes - metabolism Female Humans Intracellular Signaling Peptides and Proteins Lipids Lysosomes Lysosomes - drug effects Lysosomes - metabolism Male Mice Mice, Inbred BALB C Mice, Knockout Neurodegenerative Diseases Neuroglia - drug effects Neuroglia - metabolism Neurons Neurons - drug effects Neurons - metabolism Niemann-Pick Disease, Type C - genetics Niemann-Pick Disease, Type C - metabolism Proteins - genetics Proteins - metabolism
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