(NPG, 2016) Pirisinu, Laura; Di Bari, Michele A.; D’Agostino, Claudia; Marcon, Stefano; Riccardi, Geraldina; Poleggi, Anna; Cohen, Mark L.; Appleby, Brian S.; Gambetti, Pierluigi; Ghetti, Bernardino; Agrimi, Umberto; Nonno, Romolo; Department of Pathology & Laboratory Medicine, IU School of Medicine
Gerstmann-Sträussler-Scheinker disease (GSS) is an inherited neurodegenerative disorder associated with mutations in the prion protein gene and accumulation of misfolded PrP with protease-resistant fragments (PrPres) of 6–8 kDa.