Transmissible mink encephalopathy (TME) is a progressive and fatal neurodegenerative disease that affects ranched mink (Neovison (Mustela) vison). Most or all of the adult animals on a ranch may be affected, and once an animal becomes symptomatic, death is inevitable. This disease is still poorly understood. It is very rare, with only a few outbreaks reported in the U.S. and other countries. Ou...

Prion diseases, or transmissible spongiform encephalopathies, include Creutzfeldt-Jakob disease (CJD) in humans and scrapie and bovine spongiform encephalopathy (BSE) in animals. These neurodegenerative diseases are invariably fatal and can be transmitted by inoculation or dietary exposure. They are associated with the accumulation of an altered, disease-associated form of the normal prion prot...

List of abbreviations Alzheimer’s Disease, AD; Amyloid Precursor Protein, APP; Beta-Amyloid, Aβ; Bovine Spongiform Encephalopathy, BSE; Creutzfeldt-Jakob Disease, CJD; Central Nervous System, CNS; Immunofluorescence, IF; Immunoprecipitation, IP; Enzyme Linked Immunosorbent Assay, ELISA; Huntingtin, Htt; Huntington’s Disease, HD; Immunohistochemistry, IHC; Luminescent Conjugated Oligothiophenes,...

The transmissible spongiform encephalopathies include human diseases such as Creutzfeldt-Jakob disease (CJD) and kuru as well as animal diseases such as scrapie and bovine spongiform encephalopathy (BSE). The emergence of variant CJD, which is causally related to BSE, has generated much interest in the development of rapid and sensitive diagnostic tests for the pre-mortem diagnosis of CJD. In 1...

Public and animal health controls to limit human exposure to animal prions are focused on bovine spongiform encephalopathy (BSE), but other prion strains in ruminants may also have zoonotic potential. One example is atypical/Nor98 scrapie, which evaded statutory diagnostic methods worldwide until the early 2000s. To investigate whether sheep infected with scrapie prions could be another source ...

BACKGROUND The cause of the bovine spongiform encephalopathy (BSE) epidemic in the United Kingdom (UK) was the inclusion of contaminated meat and bone meal in the protein rations fed to cattle. Those rations were not restricted to cattle but were also fed to other livestock including farmed and free living deer. Although there are no reported cases to date of natural BSE in European deer, BSE h...

Amino acid replacements encoded by the prion protein gene (PRNP) have been associated with transmissible and hereditary spongiform encephalopathies in mammalian species. However, an association between bovine spongiform encephalopathy (BSE) and bovine PRNP exon 3 has not been detected. Moreover, little is currently known regarding the mechanisms of evolution influencing the bovine PRNP gene. Th...

The appearance of bovine spongiform encephalopathy (BSE) as a new disease of cattle in 1985-1987 increased worldwide interest in various aspects of human and animal spongiform encephalopathies. In the United States, a part of the surveillance effort has been directed toward prospective examination of bovine brain specimens for lesions of BSE. One focus area has been to obtain specimens from cat...

Distinct prion strains often exhibit different incubation periods and patterns of neuropathological lesions. Strain characteristics are generally retained upon intraspecies transmission, but may change on transmission to another species. We investigated the inactivation of two related prions strains: BSE prions from cattle and mouse-passaged BSE prions, termed 301V. Inactivation was manipulated...