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L-leucine and SPNS1 coordinately ameliorate dysfunction of autophagy in mouse and human Niemann-Pick type C disease

Lysosomal storage disorders are characterized by progressive accumulation of undigested macromolecules within the cell due to lysosomal dysfunction. 573C10 is a Schwann cell line derived from a mouse model of Niemann-Pick type C disease-1, NPC (−/−). Under serum-starved conditions, NPC (−/−) cells m...

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Detalles Bibliográficos
Autores principales:	Yanagisawa, Hiroko, Ishii, Tomohiro, Endo, Kentaro, Kawakami, Emiko, Nagao, Kazuaki, Miyashita, Toshiyuki, Akiyama, Keiko, Watabe, Kazuhiko, Komatsu, Masaaki, Yamamoto, Daisuke, Eto, Yoshikatsu
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Nature Publishing Group UK 2017
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5698481/ https://www.ncbi.nlm.nih.gov/pubmed/29162837 http://dx.doi.org/10.1038/s41598-017-15305-9

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