Article
Alexander Disease Modeling in Zebrafish: An In Vivo System Suitable to Perform Drug Screening.
Genes - 11 Dec 2020
Candiani Simona, Carestiato Silvia, Mack Andreas F, Bani Daniele, Bozzo Matteo, Obino Valentina, Ori Michela, Rosamilia Francesca, De Sarlo Miriam, Pestarino Mario, Ceccherini Isabella, Bachetti Tiziana
Abstract excerpt
Alexander disease (AxD) is a rare astrogliopathy caused by heterozygous mutations, either inherited or arising de novo, on the glial fibrillary acid protein (GFAP) gene (17q21). Mutations in the GFAP gene make the protein prone to forming aggregates which, together with heat-shock protein 27 (HSP27), αB-crystallin, ubiquitin, and proteasome, contribute to form Rosenthal fibers causing a toxic effect on the cell....
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