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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Autors principals: Kwiatkowska, Katarzyna, Marszałek–Sadowska, Ewelina, Traczyk, Gabriela, Koprowski, Piotr, Musielak, Małgorzata, Ługowska, Agnieszka, Kulma, Magdalena, Grzelczyk, Anna, Sobota, Andrzej
Format: Artigo
Idioma:Inglês
Publicat: BioMed Central 2014
Matèries:
Research
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC4005833/
https://ncbi.nlm.nih.gov/pubmed/24775609
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/1750-1172-9-64
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