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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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| Main Authors: | , , |
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| Format: | Artigo |
| Sprog: | Inglês |
| Udgivet: |
2011
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| Fag: | |
| Online adgang: | 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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