Book Chapter

Molecular Subtyping of PrPres in Human Sporadic CJD Brain Tissue

GM Klug, V Lewis, SJ Collins

PRIONS: METHODS AND PROTOCOLS | Methods in Molecular Biology | HUMANA PRESS INC | Published : 2017


Across the spectrum of sporadic human prion diseases (also known as transmissible spongiform encephalopathies: TSE), there is considerable phenotypic diversity. Cumulative scientific evidence supports that prions, the infectious agents of prion diseases, are constituted predominantly, if not exclusively, by misfolded, typically protease-resistant, disease-associated isoforms of the prion protein (PrPres). Consequently, tissue deposition of PrPres is considered a hallmark of prion disease pathology, and this can be visualized by Western blotting after tissue homogenization and treatment with proteinases, particularly proteinase K (PK). Indeed, Western blot profiles of PrPres are utilized as o..

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