Alexander disease mutations differentially sensitize glial fibrillary acidic protein (GFAP) to posttranslational modifications and network disruption by oxidants.

Abstract excerpt

The type III intermediate filament protein glial fibrillary acidic protein (GFAP) plays a key role in astrocyte and brain homeostasis. Mutations in GFAP can result in Alexander's disease (AxD), a severe neurodegenerative disease. Studies on AxD models indicate that oxidative stress may be an important pathogenic factor. Cellular expression of certain GFAP AxD mutants can provoke oxidative stress and contribute to...

Topics

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• Alexander Disease
• GFAP gene
• Glial Fibrillary Acidic Protein
• Hydrogen Peroxide
• Mutation