FcRγ-dependent immune activation initiates astrogliosis during the asymptomatic phase of Sandhoff disease model mice

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(−...

Ausführliche Beschreibung

Gespeichert in:
Bibliographische Detailangaben
Veröffentlicht in:Sci Rep
Hauptverfasser: 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
Format: Artigo
Sprache:Inglês
Veröffentlicht: Nature Publishing Group 2017
Schlagworte:
Article
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5234013/
https://ncbi.nlm.nih.gov/pubmed/28084424
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/srep40518
Tags: Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!

Ähnliche Einträge