Alexander Disease Mutations Produce Cells with Coexpression of Glial Fibrillary Acidic Protein and NG2 in Neurosphere Cultures and Inhibit Differentiation into Mature Oligodendrocytes

Alexander Disease Mutations Produce Cells with Coexpression of Glial Fibrillary Acidic Protein and NG2 in Neurosphere Cultures and Inhibit Differentiation into Mature Oligodendrocytes

BackgroundAlexander disease (AxD) is a rare disease caused by mutations in the gene encoding glial fibrillary acidic protein (GFAP). The disease is characterized by presence of GFAP aggregates in the cytoplasm of astrocytes and loss of myelin.ObjectivesDetermine the effect of AxD-related mutations o...

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Bibliographic Details
Main Authors: Ulises Gómez-Pinedo, Maria Salomé Sirerol-Piquer, María Durán-Moreno, José Manuel García-Verdugo, Jorge Matias-Guiu
Format: Article
Language:English
Published: Frontiers Media S.A. 2017-06-01
Series:Frontiers in Neurology
Subjects:
Alexander disease
glial fibrillary acidic protein
NG2
neurospheres
oligodendrocyte precursors
cathepsin
Online Access:http://journal.frontiersin.org/article/10.3389/fneur.2017.00255/full

Internet

http://journal.frontiersin.org/article/10.3389/fneur.2017.00255/full

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