Regulation of Sterol Transport between Membranes and NPC2

Niemann-Pick disease type C (NPC) is caused by defects in either the NPC1 or NPC2 gene and is characterized by accumulation of cholesterol and glycolipids in the late endosome/lysosome compartment. NPC2 is an intralysosomal protein that binds cholesterol in vitro. Previous studies demonstrated rapid...

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Veröffentlicht in:	Biochemistry (Easton) 2008-10, Vol.47 (42), p.11134-11143
Hauptverfasser:	Xu, Zhi, Farver, William, Kodukula, Sarala, Storch, Judith
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Biological Transport, Active - drug effects Carrier Proteins - chemistry Carrier Proteins - genetics Carrier Proteins - metabolism Cattle Cholesterol - metabolism Dolichol - pharmacology Endosomes - metabolism Ergosterol - analogs & derivatives Ergosterol - metabolism Fluorescent Dyes Gangliosides - pharmacology Glycoproteins - chemistry Glycoproteins - genetics Glycoproteins - metabolism Humans In Vitro Techniques Intracellular Membranes - metabolism Kinetics Lysosomes - metabolism Niemann-Pick Disease, Type C - genetics Niemann-Pick Disease, Type C - metabolism Recombinant Proteins - chemistry Recombinant Proteins - genetics Recombinant Proteins - metabolism Spectrometry, Fluorescence Spectroscopy, Fourier Transform Infrared Sterols - metabolism Tryptophan - chemistry Vesicular Transport Proteins - chemistry Vesicular Transport Proteins - genetics Vesicular Transport Proteins - metabolism
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