Article
Genetic human prion disease modelled in PrP transgenic Drosophila.
The Biochemical journal - 20 Sept 2017
Thackray Alana M, Cardova Alzbeta, Wolf Hanna, Pradl Lydia, Vorberg Ina, Jackson Walker S, Bujdoso Raymond
Abstract excerpt
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 PrPSc, an abnormal isomer of the normal host protein PrPC, in the brain of affected individuals. PrPSc is the principal component of the transmissible neurotoxic prion agent. It is important to...
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