Alexander disease GFAP R239C mutant shows increased susceptibility to lipoxidation and elicits mitochondrial dysfunction and oxidative stress

Alexander disease GFAP R239C mutant shows increased susceptibility to lipoxidation and elicits mitochondrial dysfunction and oxidative stress

Alexander disease is a fatal neurological disorder caused by mutations in the intermediate filament protein Glial Fibrillary Acidic Protein (GFAP), which is key for astrocyte homeostasis. These mutations cause GFAP aggregation, astrocyte dysfunction and neurodegeneration. Remarkably, most of the kno...

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Bibliographic Details
Main Authors: Álvaro Viedma-Poyatos, Patricia González-Jiménez, María A. Pajares, Dolores Pérez-Sala
Format: Article
Language:English
Published: Elsevier 2022-09-01
Series:Redox Biology
Subjects:
Neurodegeneration
Mitochondrial dysfunction
GFAP aggregation
Protein lipoxidation
Oxidative damage
AxD cellular model
Online Access:http://www.sciencedirect.com/science/article/pii/S2213231722001872

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http://www.sciencedirect.com/science/article/pii/S2213231722001872

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