Project description:Selective neuronal vulnerability is a common, yet poorly understood characteristic of neurodegenerative diseases and is particularly prominent in familial prion diseases, such as Creutzfeldt-Jakob disease (CJD) and fatal familial insomnia (FFI), where different mutations in the prion protein manifest as neuropathologically distinct diseases. To determine how presence of mutant prion protein influences gene expression at pre-symptomatic stages, we used RiboTag to isolate cell type-specific, translating RNA from GABAergic, glutamatergic, somatostatin- (SST) and parvalbumin-expressing neurons of 9-month-old knock-in mice of CJD and FFI.

2022-09-21 | GSE198063 | GEO

Fatal familial insomnia: mitochondrial and protein synthesis machinery decline in the mediodorsal thalamus.

Project description: Not available

| S-EPMC8029211 | biostudies-literature

Prion-induced amyloid heart disease with high blood infectivity in transgenic mice.

Project description: Not available

| S-EPMC1820586 | biostudies-literature

An in vivo 11C-PK PET study of microglia activation in Fatal Familial Insomnia.

Project description: Not available

| S-EPMC5771322 | biostudies-literature

Correction: Fatal Prion Disease in a Mouse Model of Genetic E200K Creutzfeldt-Jakob Disease.

Project description: Not available