Alterations in ROS Activity and Lysosomal pH Account for Distinct Patterns of Macroautophagy in LINCL and JNCL Fibroblasts

Neuronal Ceroid Lipofuscinoses (NCL) are lysosomal storage disorders characterized by the accumulation of lipofuscin within lysosomes. Late infantile (LINCL) and juvenile (JNCL) are their most common forms and are caused by loss-of-function mutations in tripeptidyl peptidase 1 (TPP1), a lysosomal en...

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Detalles Bibliográficos
Autores principales: Vidal-Donet, José Manuel, Cárcel-Trullols, Jaime, Casanova, Bonaventura, Aguado, Carmen, Knecht, Erwin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2013
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3567113/
https://www.ncbi.nlm.nih.gov/pubmed/23408996
http://dx.doi.org/10.1371/journal.pone.0055526

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