Genetic human prion disease modelled in PrP transgenic Drosophila.

ABSTRACT: Inherited human prion diseases, such as fatal familial insomnia (FFI) and familial Creutzfeldt-Jakob disease (fCJD), are associated with autosomal dominant mutations in the human prion protein gene PRNP and accumulation of PrP^Sc, an abnormal isomer of the normal host protein PrP^C, in the brain of affected individuals. PrP^Sc is the principal component of the transmissible neurotoxic prion agent. It is important to identify molecular pathways and cellular processes that regulate prion formation and prion-induced neurotoxicity. This will allow identification of possible therapeutic interventions for individuals with, or at risk from, genetic human prion disease. Increasingly, Drosophila has been used to model human neurodegenerative disease. An i

SUBMITTER: Thackray AM 

PROVIDER: S-EPMC5606059 | biostudies-literature | 2017 Sep

REPOSITORIES: biostudies-literature