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GM2 ganglioside accumulation causes neuroinflammation and behavioral alterations in a mouse model of early onset Tay-Sachs disease

BACKGROUND: Tay-Sachs disease (TSD), a type of GM2-gangliosidosis, is a progressive neurodegenerative lysosomal storage disorder caused by mutations in the α subunit of the lysosomal β-hexosaminidase enzyme. This disease is characterized by excessive accumulation of GM2 ganglioside, predominantly in...

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Detalles Bibliográficos
Autores principales: Demir, Seçil Akyıldız, Timur, Zehra Kevser, Ateş, Nurselin, Martínez, Luis Alarcón, Seyrantepe, Volkan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7504627/
https://www.ncbi.nlm.nih.gov/pubmed/32951593
http://dx.doi.org/10.1186/s12974-020-01947-6