Axonopathy and Reduction of Membrane Resistance: Key Features in a New Murine Model of Human G<sub>M1</sub>-Gangliosidosis

Axonopathy and Reduction of Membrane Resistance: Key Features in a New Murine Model of Human G<sub>M1</sub>-Gangliosidosis

G<sub>M1</sub>-gangliosidosis is caused by a reduced activity of β-galactosidase (<i>Glb1</i>), resulting in intralysosomal accumulations of G<sub>M1</sub>. The aim of this study was to reveal the pathogenic mechanisms of G<sub>M1</sub>-gangliosidosis...

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Bibliographic Details
Main Authors: Deborah Eikelberg, Annika Lehmbecker, Graham Brogden, Witchaya Tongtako, Kerstin Hahn, Andre Habierski, Julia B. Hennermann, Hassan Y. Naim, Felix Felmy, Wolfgang Baumgärtner, Ingo Gerhauser
Format: Article
Language:English
Published: MDPI AG 2020-04-01
Series:Journal of Clinical Medicine
Subjects:
astrogliosis
axonopathy
β-galactosidase deficiency
electrophysiology
G<sub>M1</sub>-gangliosidosis
knockout mouse model
Online Access:https://www.mdpi.com/2077-0383/9/4/1004

Internet

https://www.mdpi.com/2077-0383/9/4/1004

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