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Murine neural stem cells model Hunter disease in vitro: glial cell-mediated neurodegeneration as a possible mechanism involved

Mucopolysaccharidosis type II (MPSII or Hunter Syndrome) is a lysosomal storage disorder caused by the deficit of iduronate 2-sulfatase (IDS) activity and characterized by progressive systemic and neurological impairment. As the early mechanisms leading to neuronal degeneration remain elusive, we ch...

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Detalles Bibliográficos
Autores principales: Fusar Poli, E, Zalfa, C, D'Avanzo, F, Tomanin, R, Carlessi, L, Bossi, M, Rota Nodari, L, Binda, E, Marmiroli, P, Scarpa, M, Delia, D, Vescovi, A L, De Filippis, L
Formato: Artigo
Lenguaje:Inglês
Publicado: Nature Publishing Group 2013
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Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC3847312/
https://ncbi.nlm.nih.gov/pubmed/24201805
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/cddis.2013.430
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