Impairment of chaperone-mediated autophagy leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosis

Metabolite accumulation in lysosomal storage disorders (LSDs) results in impaired cell function and multi-systemic disease. Although substrate reduction and lysosomal overload-decreasing therapies can ameliorate disease progression, the significance of lysosomal overload-independent mechanisms in th...

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Detalles Bibliográficos
Autores principales: Napolitano, Gennaro, Johnson, Jennifer L, He, Jing, Rocca, Celine J, Monfregola, Jlenia, Pestonjamasp, Kersi, Cherqui, Stephanie, Catz, Sergio D
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BlackWell Publishing Ltd 2015
Materias:
Research Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4328646/
https://www.ncbi.nlm.nih.gov/pubmed/25586965
http://dx.doi.org/10.15252/emmm.201404223

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4328646/
https://www.ncbi.nlm.nih.gov/pubmed/25586965
http://dx.doi.org/10.15252/emmm.201404223

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