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Early onset effects of single substrate accumulation recapitulate major features of LSD in patient-derived lysosomes

Lysosome functions mainly rely on their ability to either degrade substrates or release them into the extracellular space. Lysosomal storage disorders (LSDs) are commonly characterized by a chronic lysosomal accumulation of different substrates, thereby causing lysosomal dysfunctions and secretion d...

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Detalles Bibliográficos
Autores principales: Scerra, Gianluca, De Pasquale, Valeria, Pavone, Luigi Michele, Caporaso, Maria Gabriella, Mayer, Andreas, Renna, Maurizio, D'Agostino, Massimo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8253970/
https://www.ncbi.nlm.nih.gov/pubmed/34258549
http://dx.doi.org/10.1016/j.isci.2021.102707