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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Detalles Bibliográficos
Autores principales: Ko, Dennis C, Milenkovic, Ljiljana, Beier, Steven M, Manuel, Hermogenes, Buchanan, JoAnn, Scott, Matthew P
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2005
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1183526/
https://www.ncbi.nlm.nih.gov/pubmed/16103921
http://dx.doi.org/10.1371/journal.pgen.0010007

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1183526/
https://www.ncbi.nlm.nih.gov/pubmed/16103921
http://dx.doi.org/10.1371/journal.pgen.0010007

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