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Impaired proteolysis underlies autophagic dysfunction in Niemann–Pick type C disease
Niemann–Pick type C disease (NPC) is a childhood onset neurodegenerative disorder arising from lipid-trafficking defects caused by mutations in the NPC1 or NPC2 gene. Marked accumulation of autophagosomes is a prominent feature of NPC cells, yet a detailed understanding of the disease-associated alt...
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| Main Authors: | , , , |
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| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
Oxford University Press
2012
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| Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC3607480/ https://ncbi.nlm.nih.gov/pubmed/22872701 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/dds324 |
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