Distinct patterns of prion strain deposition and toxicity in a novel whole brain organotypic slice culture system.

ABSTRACT: Prion diseases are fatal transmissible neurodegenerative diseases that affect many mammals, including humans, caused by the templated misfolding of the prion protein. Different conformations of misfolded prions can occur, leading to distinct disease phenotypes or strains and the accumulation of prions in distinct brain regions. How prion structure influences this brain tropism is not clear, but the transmissible nature of prion diseases has allowed for the development of ex vivo brain slice models of disease. To date, work has been done in cerebellar cultures, but prion diseases are known to differentially affect many other brain regions. We have adapted this approach to a coronally sliced whole brain organotypic culture and demonstrate distinct profiles of cytotoxicity and neuronal loss u

SUBMITTER: Pineau H 

PROVIDER: S-EPMC11805914 | biostudies-literature | 2025 Feb

REPOSITORIES: biostudies-literature