Extracellular Prion Protein Aggregates in Nine Gerstmann–Sträussler–Scheinker Syndrome Subjects with Mutation P102L: A Micromorphological Study and Comparison with Literature Data

• Published in: International journal of molecular sciences Vol. 22; no. 24; p. 13303
• Main Authors: Jankovska, Nikol, Matej, Radoslav, Olejar, Tomas
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 10.12.2021; MDPI
• Subjects: Adult; Age; Aged; Alzheimer's disease; Comorbidity; Female; Gerstmann-Straussler-Scheinker Disease - genetics; Gerstmann-Straussler-Scheinker Disease - metabolism; Gerstmann-Straussler-Scheinker Disease - pathology; Humans; Male; Middle Aged; Monoclonal antibodies; Mutation; Mutation, Missense; Pathology; Prion Proteins - genetics; Prion Proteins - metabolism; Protein Aggregation, Pathological - genetics; Protein Aggregation, Pathological - metabolism; Protein Aggregation, Pathological - pathology; Proteins; Gerstmann–Sträussler–Scheinker syndrome; co-expression; plaques; PrP

Gerstmann–Sträussler–Scheinker syndrome (GSS) is a hereditary neurodegenerative disease characterized by extracellular aggregations of pathological prion protein (PrP) forming characteristic plaques. Our study aimed to evaluate the micromorphology and protein composition of these plaques in relation...