From the European Society for Clinical Virology 2022 Conference in Manchester UK, Vincent speaks with Richard Knight about prion diseases and the outbreak of bovine spongiform encephalitis that led to cases of variant Creutzfeldt-Jakob disease in humans. Host: Vincent Racaniello Guest: Richard Knight Click arrow to playDownload TWiV 950 (68 MB .mp3, 114 min)Subscribe (free): Apple Podcasts, Google Podcasts, RSS, email Become …
by Gertrud U. Rey Transmissible spongiform encephalopathies (TSEs) include a variety of fatal neurodegenerative diseases caused by infectious proteins called prions. Although prions are not viruses, their ability to self-propagate without a nucleic acid intermediate has always fascinated virologists, causing them to adopt prions into their repertoire of pathogenic agents. Common TSEs comprise scrapie in …
A sensitive and specific blood test has been developed that could be used to limit the risk of transmission of prion disease through the blood supply (link to papers one and two). Prion diseases, also known as spongiform encephalopathies, are uniformly fatal, chronic degenerative neurological diseases caused by misfolding of a cellular protein, PrPC. Transmissible …
Spongiform encephalopathies are neurodegenerative diseases caused by misfolding of normal cellular prion proteins. A 2014 case of variant Creutzfeldt-Jacob prion disease in the United States was probably caused by eating beef from animals with bovine spongiform encephalopathy (BSE), or mad cow disease. Human spongiform encephalopathies are placed into three groups: infectious, familial or genetic, and sporadic, distinguished by how the disease …
A case of prion disease acquired from contaminated beef Read More »
Transmissible spongiform encephalopathies (TSEs) are rare human neurodegenerative disorders that are caused by infectious proteins called prions. A naturally occurring variant of the human prion has been found that completely protects against the disease. A protective variant of the prion protein was discovered in the Fore people of Papua New Guinea. Beginning in the early 1900s, the prion disease kuru spread among Fore women and children as …
Chronic wasting disease (CWD) is a prion disease of cervids (deer, elk, moose). It was first detected in Wyoming and Colorado, and has since spread rapidly throughout North America (illustrated; image credit). Because prions that cause bovine spongiform encephalopathy (BSE, mad cow disease) are known to infect humans, there is concern that CWD might also cross the …
