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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...
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 |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group
2013
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3847312/ https://www.ncbi.nlm.nih.gov/pubmed/24201805 http://dx.doi.org/10.1038/cddis.2013.430 |
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