Protease-sensitive prionopathy

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  Bovine spongiform encephalopathy

  Bovine spongiform encephalopathy (BSE), commonly known as mad-cow disease (MCD), is a fatal, neurodegenerative disease in cattle, that causes a spongy degeneration in the brain and spinal cord. BSE has a long incubation period, about 4 years, usually affecting adult cattle at a peak age onset of...
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  Scrapie

  Scrapie is a fatal, degenerative disease that affects the nervous systems of sheep and goats. It is one of several transmissible spongiform encephalopathies (TSEs), which are related to bovine spongiform encephalopathy (BSE or "mad cow disease") and chronic wasting disease of deer. Like other...
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  Chronic wasting disease

  Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) of mule deer, whitetailed deer, elk (wapiti), and moose. TSEs are caused by unusual infectious agents known as prions. To date, CWD has been found mainly in cervids (members of the deer family). First recognized as a...

• Kuru

  Kuru is an incurable degenerative neurological disorder (brain disease) that is a type of transmissible spongiform encephalopathy found in humans. Kuru is believed to be caused by prions and is related to Creutzfeldt-Jakob disease. It is best known for the epidemic that occurred in Papua New Guinea...

• Fatal familial insomnia

  Fatal familial insomnia (FFI) is a very rare autosomal dominant inherited prion disease of the brain. The gene mutation responsible has been found in just 50 families worldwide; if only one parent has the gene, the offspring have a 50% chance of inheriting it and developing the disease. The disease...

• Monkeypox

  Monkeypox is an exotic infectious disease caused by the monkeypox virus. The disease was first identified in laboratory monkeys, thus providing it with its name, but in its natural state it seems to infect rodents more than primates. The disease is most prevalent in Central and West Africa, but an...

• Ure2p

  Ure2p is a yeast protein that represses transcription of genes involved in nitrogen catabolism. It specifically regulates the utilization of poor nitrogen sources in the presence of preferred nutrients such as ammonia or glutamine. Ure2p is one of the few yeast proteins that are known to be prions....

• Sup35p

  Sup35p is the Saccharomyces cerevisiae (a yeast) eukaryotic translation termination factor. More specifically, it is the yeast eukaryotic release factor 3 (eRF3), which forms the translation termination complex with eRF1 (Sup45p in yeast). This complex recognizes and catalyzes the release of the...

• Feline spongiform encephalopathy

  Feline spongiform encephalopathy is a disease that affects the brains and livers of felines. It is caused by proteins called prions. Feline spongiform encephalopathy (FSE) is a prion disease thought to be related to Bovine spongiform encephalopathy (BSE). This disease is known to affect domestic...

• Transmissible mink encephalopathy

  Transmissible mink encephalopathy (TME) is rare sporadic disease that affects the central nervous system of ranch-raised mink. It is classified as a transmissible spongiform encephalopathy, believed to be caused by proteins called prions. This disease is only known to affect adult mink. This...