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Activation of Stress Kinases in the Brain of Mucopolysaccharidosis IIIB mice
The accumulation of heparan sulfate (HS) in lysosomes is the primary consequence of the enzyme defect (α-N-acetylglucosaminidase) in mucopolysaccharidosis type IIIB. This accumulation triggers a cascade of pathological events that progressively leads to CNS pathology. Here we examined the activation...
Autores principales: | Cecere, Francesca, Di Domenico, Carmela, Di Napoli, Daniele, Boscia, Francesca, Di Natale, Paola |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wiley Subscription Services, Inc., A Wiley Company
2011
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3166654/ https://www.ncbi.nlm.nih.gov/pubmed/21618584 http://dx.doi.org/10.1002/jnr.22674 |
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