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Molecular Pathways for Intracellular Cholesterol Accumulation: Common Pathogenic Mechanisms in Niemann-Pick Disease Type C and Cystic Fibrosis

It has been less than two decades since the underlying genetic defects in Niemann-Pick disease Type C were first identified. These defects impair function of two proteins with a direct role in lipid trafficking, resulting in deposition of free cholesterol within late endosomal compartments and a mul...

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Autores principales: Cianciola, Nicholas L., Carlin, Cathleen R., Kelley, Thomas J.
Formato: Artigo
Lenguaje:Inglês
Publicado: 2011
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Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3192251/
https://ncbi.nlm.nih.gov/pubmed/21924233
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.abb.2011.08.012
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