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Defective Autophagy, Mitochondrial Clearance and Lipophagy in Niemann-Pick Type B Lymphocytes

Niemann-Pick disease type A (NP-A) and type B (NP-B) are lysosomal storage diseases (LSDs) caused by sphingomyelin accumulation in lysosomes relying on reduced or absent acid sphingomyelinase. A considerable body of evidence suggests that lysosomal storage in many LSD impairs autophagy, resulting in...

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Detalles Bibliográficos
Autores principales:	Canonico, Barbara, Cesarini, Erica, Salucci, Sara, Luchetti, Francesca, Falcieri, Elisabetta, Di Sario, Gianna, Palma, Fulvio, Papa, Stefano
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Public Library of Science 2016
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087958/ https://www.ncbi.nlm.nih.gov/pubmed/27798705 http://dx.doi.org/10.1371/journal.pone.0165780

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