11 articles
Antiprion drug research
Antiprion drug research seeks treatments for fatal prion diseases; no approved therapy exists. Leading approaches lower prion protein via ASOs and siRNA, now in first human trials (ION717, PRiSM, ALN-6457).
Bovine spongiform encephalopathy
Bovine spongiform encephalopathy (BSE, mad cow disease) is a fatal prion disease of cattle, classical form feed-borne and zoonotic causing vCJD; atypical H/L-type sporadic; diagnosed post-mortem; WOAH-listed.
Chronic wasting disease
Chronic wasting disease (CWD) is a fatal prion disease of deer, elk, moose, and reindeer, first recognized in Colorado in 1967, now in 37 US states and five Canadian provinces, with no reported human cases.
Fatal familial insomnia
Fatal familial insomnia (FFI) is a rare inherited prion disease caused by a PRNP gene mutation, producing untreatable insomnia, autonomic failure, and death within about one to two years.
Fungal prions
Fungal prions are heritable protein conformations in fungi, passed through cytoplasm rather than DNA, whose study gave the first genetic proof that proteins can be infectious.
Gerstmann–Sträussler–Scheinker syndrome
Gerstmann–Sträussler–Scheinker syndrome (GSS) is a rare autosomal dominant hereditary prion disease caused by PRNP mutations, first described in 1936, marked by multicentric PrP amyloid plaques, progressive cerebellar ataxia, and later dementia.
Major prion protein
Major prion protein (PrP, PRNP gene, chromosome 20) is a 253-amino-acid GPI-anchored cell-surface glycoprotein whose misfolded isoform PrPSc causes CJD, GSS, FFI, scrapie, and BSE; normal function unknown.
Prion
Prion (proteinaceous infectious particle): infectious agent of misfolded PrPSc protein without nucleic acid, causing fatal diseases such as CJD, BSE, scrapie, and CWD; replicates by templating PrPC conversion; no treatment exists.
Protein misfolding cyclic amplification
Protein misfolding cyclic amplification (PMCA) is a laboratory technique, first described in 2001 by Saborio and colleagues, that exponentially amplifies minute quantities of misfolded prion protein through incubation and sonication cycles.
Scrapie
Scrapie is a fatal prion disease of the central nervous system of sheep and goats, the earliest known TSE, first reported in Great Britain in 1732.
Transmissible spongiform encephalopathy
Transmissible spongiform encephalopathies (TSEs), also called prion diseases, are rare, invariably fatal degenerative brain disorders caused by misfolded prion protein; human forms include Creutzfeldt-Jakob disease, kuru, and fatal familial insomnia.