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Loss of the batten disease protein CLN3 leads to mis-trafficking of M6PR and defective autophagic-lysosomal reformation

Batten disease, one of the most devastating types of neurodegenerative lysosomal storage disorders, is caused by mutations in CLN3. Here, we show that CLN3 is a vesicular trafficking hub connecting the Golgi and lysosome compartments. Proteomic analysis reveals that CLN3 interacts with several endo-...

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Detalles Bibliográficos
Autores principales: Calcagni’, Alessia, Staiano, Leopoldo, Zampelli, Nicolina, Minopoli, Nadia, Herz, Niculin J., Di Tullio, Giuseppe, Huynh, Tuong, Monfregola, Jlenia, Esposito, Alessandra, Cirillo, Carmine, Bajic, Aleksandar, Zahabiyon, Mahla, Curnock, Rachel, Polishchuk, Elena, Parkitny, Luke, Medina, Diego Luis, Pastore, Nunzia, Cullen, Peter J., Parenti, Giancarlo, De Matteis, Maria Antonietta, Grumati, Paolo, Ballabio, Andrea
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10317969/
https://www.ncbi.nlm.nih.gov/pubmed/37400440
http://dx.doi.org/10.1038/s41467-023-39643-7

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