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Axonopathy and Reduction of Membrane Resistance: Key Features in a New Murine Model of Human G(M1)-Gangliosidosis

G(M1)-gangliosidosis is caused by a reduced activity of β-galactosidase (Glb1), resulting in intralysosomal accumulations of G(M1). The aim of this study was to reveal the pathogenic mechanisms of G(M1)-gangliosidosis in a new Glb1 knockout mouse model. Glb1(−/−) mice were analyzed clinically, histo...

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Detalhes bibliográficos
Publicado no:J Clin Med
Main Authors: Eikelberg, Deborah, Lehmbecker, Annika, Brogden, Graham, Tongtako, Witchaya, Hahn, Kerstin, Habierski, Andre, Hennermann, Julia B., Naim, Hassan Y., Felmy, Felix, Baumgärtner, Wolfgang, Gerhauser, Ingo
Formato: Artigo
Idioma:Inglês
Publicado em: MDPI 2020
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7230899/
https://ncbi.nlm.nih.gov/pubmed/32252429
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.3390/jcm9041004
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