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

Abstract 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, predomin...

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Bibliographic Details
Main Authors: Seçil Akyıldız Demir, Zehra Kevser Timur, Nurselin Ateş, Luis Alarcón Martínez, Volkan Seyrantepe
Format: Article
Language:English
Published: BMC 2020-09-01
Series:Journal of Neuroinflammation
Subjects:
Online Access:http://link.springer.com/article/10.1186/s12974-020-01947-6