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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...
Autores principales: | Scerra, Gianluca, De Pasquale, Valeria, Pavone, Luigi Michele, Caporaso, Maria Gabriella, Mayer, Andreas, Renna, Maurizio, D'Agostino, Massimo |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2021
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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 |
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