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Reversal of Pathologic Lipid Accumulation in NPC1-Deficient Neurons by Drug-Promoted Release of LAMP1-Coated Lamellar Inclusions

Aging and pathologic conditions cause intracellular aggregation of macromolecules and the dysfunction and degeneration of neurons, but the mechanisms are largely unknown. Prime examples are lysosomal storage disorders such as Niemann–Pick type C (NPC) disease, where defects in the endosomal–lysosoma...

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Detalhes bibliográficos
Publicado no:J Neurosci
Main Authors: Demais, Valérie, Barthélémy, Amélie, Perraut, Martine, Ungerer, Nicole, Keime, Céline, Reibel, Sophie, Pfrieger, Frank W.
Formato: Artigo
Idioma:Inglês
Publicado em: Society for Neuroscience 2016
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6601882/
https://ncbi.nlm.nih.gov/pubmed/27466344
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.0900-16.2016
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