A unified model for the origins of spongiform degeneration and other neuropathological features in prion diseases.

ABSTRACT: Decades after their initial observation in prion-infected brain tissues, the identities of virus-like dense particles, varicose tubules, and oval bodies containing parallel bands and fibrils have remained elusive. Our recent work revealed that a phenotype of dilation of the endoplasmic reticulum (ER), most notable for the perinuclear space (PNS), contributes to spongiform degeneration. To assess the significance of this phenotype for the etiology of prion diseases, we explored whether it can be functionally linked to other neuropathological hallmarks observed in these diseases, as this would indicate it to be a central event. Having surveyed the neuropathological record and other distant literature niches, we propose a model in which pathogenic forms of the prion protein poison raft domain

SUBMITTER: Schmitt-Ulms G 

PROVIDER: S-EPMC11774453 | biostudies-literature | 2025 Jan

REPOSITORIES: biostudies-literature