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Neurodegeneration and functional impairments associated with glycogen synthase accumulation in a mouse model of Lafora disease
Lafora disease (LD) is caused by mutations in either the laforin or malin gene. The hallmark of the disease is the accumulation of polyglucosan inclusions called Lafora Bodies (LBs). Malin knockout (KO) mice present polyglucosan accumulations in several brain areas, as do patients of LD. These struc...
Autores principales: | Valles-Ortega, Jordi, Duran, Jordi, Garcia-Rocha, Mar, Bosch, Carles, Saez, Isabel, Pujadas, Lluís, Serafin, Anna, Cañas, Xavier, Soriano, Eduardo, Delgado-García, José M, Gruart, Agnès, Guinovart, Joan J |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
WILEY-VCH Verlag
2011
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3377110/ https://www.ncbi.nlm.nih.gov/pubmed/21882344 http://dx.doi.org/10.1002/emmm.201100174 |
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