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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...
| Autores principales: | , , , , , , , , , , |
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| Formato: | Online Artículo Texto |
| Lenguaje: | English |
| Publicado: |
MDPI
2020
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| Materias: | |
| Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7230899/ https://www.ncbi.nlm.nih.gov/pubmed/32252429 http://dx.doi.org/10.3390/jcm9041004 |