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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Detaylı Bibliyografya
Yayımlandı:Cell Death Dis
Asıl Yazarlar: 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
Materyal Türü: Artigo
Dil:Inglês
Baskı/Yayın Bilgisi: Nature Publishing Group 2016
Konular:
Original Article
Online Erişim:https://ncbi.nlm.nih.gov/pmc/articles/PMC5108318/
https://ncbi.nlm.nih.gov/pubmed/27512952
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/cddis.2016.231
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