Prion Strain-Specific Structure and Pathology: A View from the Perspective of Glycobiology.

ABSTRACT: Prion diseases display multiple disease phenotypes characterized by diverse clinical symptoms, different brain regions affected by the disease, distinct cell tropism and diverse PrPSc deposition patterns. The diversity of disease phenotypes within the same host is attributed to the ability of PrPC to acquire multiple, alternative, conformationally distinct, self-replicating PrPSc states referred to as prion strains or subtypes. Structural diversity of PrPSc strains has been well documented, yet the question of how different PrPSc structures elicit multiple disease phenotypes remains poorly understood. The current article reviews emerging evidence suggesting that carbohydrates in the form of sialylated N-linked glycans, which are a constitutive part of PrPSc, are important players in defini

SUBMITTER: Baskakov IV 

PROVIDER: S-EPMC6315442 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature