neurodegenerative pathway prion disease pathway KEGG:05020 PW:0000019 Prion disease - any of a group of fatal, transmissible neurodegenerative diseases caused by abnormalities of prion protein metabolism, which may result from mutations in the prion protein gene or from infection with pathogenic isoforms of the protein. Characteristics include neuronal loss, gliosis, and extensive vacuolization of the cerebral cortex. Prion diseases may be sporadic, inherited as an autosomal dominant trait, or acquired. Human diseases include Creutzfeldt-Jakob disease, Gerstmann-Strussler syndrome, fatal familial insomnia, and kuru pathway prion protein disease pathway spongiform encephalopathy pathway transmissible spongiform encephalopathy pathway