TSE

TWiV 950: Prion diseases with Richard Knight

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 …

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How prions make you sick

Transmissible spongiform encephalopathies (TSEs) are rare, but always fatal, neurodegenerative disorders of humans and other mammals. They are characterized by long incubation periods, spongiform changes in the brain associated with loss of neurons, and the absence of host responses. TSEs are caused by infectious proteins called prions. Insight into how prions cause TSEs comes from the observation that …

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Prion contamination in the emergency room

Here is a follow-up to last week’s article that described a case of variant Creutzfeldt-Jacob disease in a Texas resident caused by ingestion of BSE-contaminated beef 14 years ago. A 59 year old male patient was admitted to the trauma unit in Lancaster, PA with a self-inflicted gunshot wound to the head. There was substantial bleeding and brain tissue extrusion …

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A case of prion disease acquired from contaminated beef

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 …

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