Glial degeneration with oxidative damage drives neuronal demise in MPSII disease

Mucopolysaccharidosis type II (MPSII) is a lysosomal storage disorder due to the deficit of the iduronate 2-sulfatase (IDS) enzyme, causing progressive neurodegeneration in patients. Neural stem cells (NSCs) derived from the IDS-ko mouse can recapitulate MPSII pathogenesis in vitro. In differentiati...

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Detalles Bibliográficos
Autores principales: Zalfa, Cristina, Verpelli, Chiara, D'Avanzo, Francesca, Tomanin, Rosella, Vicidomini, Cinzia, Cajola, Laura, Manara, Renzo, Sala, Carlo, Scarpa, Maurizio, Vescovi, Angelo Luigi, De Filippis, Lidia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2016
Materias:
Original Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5108318/
https://www.ncbi.nlm.nih.gov/pubmed/27512952
http://dx.doi.org/10.1038/cddis.2016.231

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5108318/
https://www.ncbi.nlm.nih.gov/pubmed/27512952
http://dx.doi.org/10.1038/cddis.2016.231

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