Article
FcRγ-dependent immune activation initiates astrogliosis during the asymptomatic phase of Sandhoff disease model mice.
Scientific reports - 13 Jan 2017
Ogawa Yasuhiro, Sano Takafumi, Irisa Masahiro, Kodama Takashi, Saito Takahiro, Furusawa Eiri, Kaizu Katsutoshi, Yanagi Yusuke, Tsukimura Takahiro, Togawa Tadayasu, Yamanaka Shoji, Itoh Kohji, Sakuraba Hitoshi, Oishi Kazuhiko
Abstract excerpt
Sandhoff disease (SD) is caused by the loss of β-hexosaminidase (Hex) enzymatic activity in lysosomes resulting from Hexb mutations. In SD patients, the Hex substrate GM2 ganglioside accumulates abnormally in neuronal cells, resulting in neuronal loss, microglial activation, and astrogliosis. Hexb-/- mice, which manifest a phenotype similar to SD, serve as animal models for examining the pathophysiology of SD....
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