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Visualization of cholesterol deposits in lysosomes of Niemann-Pick type C fibroblasts using recombinant perfringolysin O

BACKGROUND: Niemann-Pick disease type C (NPC) is caused by defects in cholesterol efflux from lysosomes due to mutations of genes coding for NPC1 and NPC2 proteins. As a result, massive accumulation of unesterified cholesterol in late endosomes/lysosomes is observed. At the level of the organism the...

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Detalles Bibliográficos
Autores principales:	Kwiatkowska, Katarzyna, Marszałek–Sadowska, Ewelina, Traczyk, Gabriela, Koprowski, Piotr, Musielak, Małgorzata, Ługowska, Agnieszka, Kulma, Magdalena, Grzelczyk, Anna, Sobota, Andrzej
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	BioMed Central 2014
Materias:	Research
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4005833/ https://www.ncbi.nlm.nih.gov/pubmed/24775609 http://dx.doi.org/10.1186/1750-1172-9-64

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