Article
Elevated GFAP isoform expression promotes protein aggregation and compromises astrocyte function.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 May 2021
Lin Ni-Hsuan, Yang Ai-Wen, Chang Chih-Hsuan, Perng Ming-Der
Abstract excerpt
Alexander disease (AxD) caused by mutations in the coding region of GFAP is a neurodegenerative disease characterized by astrocyte dysfunction, GFAP aggregation, and Rosenthal fiber accumulation. Although how GFAP mutations cause disease is not fully understood, Rosenthal fibers could be induced by forced overexpression of human GFAP and this could be lethal in mice implicate that an increase in GFAP levels is...
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