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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Vydáno v:J Clin Med
Hlavní autoři: Eikelberg, Deborah, Lehmbecker, Annika, Brogden, Graham, Tongtako, Witchaya, Hahn, Kerstin, Habierski, Andre, Hennermann, Julia B., Naim, Hassan Y., Felmy, Felix, Baumgärtner, Wolfgang, Gerhauser, Ingo
Médium: Artigo
Jazyk:Inglês
Vydáno: MDPI 2020
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On-line přístup: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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