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Cell-Autonomous Death of Cerebellar Purkinje Neurons with Autophagy in Niemann-Pick Type C Disease

Niemann-Pick type C is a neurodegenerative lysosomal storage disorder caused by mutations in either of two genes, npc1 and npc2. Cells lacking Npc1, which is a transmembrane protein related to the Hedgehog receptor Patched, or Npc2, which is a secreted cholesterol-binding protein, have aberrant orga...

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Detalhes bibliográficos
Main Authors: Ko, Dennis C, Milenkovic, Ljiljana, Beier, Steven M, Manuel, Hermogenes, Buchanan, JoAnn, Scott, Matthew P
Formato: Artigo
Idioma:Inglês
Publicado em: Public Library of Science 2005
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC1183526/
https://ncbi.nlm.nih.gov/pubmed/16103921
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pgen.0010007
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