Bachetti, Tiziana and Di Zanni, Eleonora and Balbi, Pietro and Ravazzolo, Roberto and Sechi, Gianpietro and Ceccherini, Isabella (2012) Beneficial effects of curcumin on GFAP filament organization and down-regulation of GFAP expression in an in vitro model of Alexander disease. Experimental Cell Research, Vol. 318 (15), p. 1844-1854. eISSN 1090-2422. Article.
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Heterozygous mutations of the GFAP gene are responsible for Alexander disease, a neurodegenerative disorder characterized by intracytoplasmic Rosenthal fibers (RFs) in dystrophic astrocytes. In vivo and in vitro models have shown co-localization of mutant GFAP proteins with the small heat shock proteins (sHSPs) HSP27 and alphaB-crystallin, ubiquitin and proteasome components. Results reported by several recent studies agree on ascribing an altered cytoskeletal pattern to mutant GFAP proteins, an effect which induces mutant proteins accumulation, leading to impaired proteasome function and autophagy induction.
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