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Metabolic Enzyme Alterations and Astrocyte Dysfunction in a Murine Model of Alexander Disease With Severe Reactive Gliosis

Alexander disease (AxD) is a rare and fatal neurodegenerative disorder caused by mutations in the gene encoding glial fibrillary acidic protein (GFAP). In this report, a mouse model of AxD (GFAP(Tg);Gfap(+/R236H)) was analyzed that contains a heterozygous R236H point mutation in murine Gfap as well...

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Detalles Bibliográficos
Autores principales: Heaven, Michael R., Herren, Anthony W., Flint, Daniel L., Pacheco, Natasha L., Li, Jiangtao, Tang, Alice, Khan, Fatima, Goldman, James E., Phinney, Brett S., Olsen, Michelle L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Biochemistry and Molecular Biology 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8717607/
https://www.ncbi.nlm.nih.gov/pubmed/34808356
http://dx.doi.org/10.1016/j.mcpro.2021.100180