Article
Activation of Src family kinase ameliorates secretory trafficking in mutant prion protein cells.
The Journal of biological chemistry - 1 Jan 2000
Restelli Elena, Capone Vanessa, Pozzoli Manuela, Ortolan Davide, Quaglio Elena, Corbelli Alessandro, Fiordaliso Fabio, Beznoussenko Galina V, Artuso Vladimiro, Roiter Ignazio, Sallese Michele, Chiesa Roberto
Abstract excerpt
Fatal familial insomnia (FFI), genetic Creutzfeldt-Jakob disease (gCJD), and Gerstmann-Sträussler-Scheinker (GSS) syndrome are neurodegenerative disorders linked to prion protein (PrP) mutations. The pathogenic mechanisms are not known, but increasing evidence points to mutant PrP misfolding and retention in the secretory pathway. We previously found that the D178N/M129 mutation associated with FFI accumulates in...
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