PRIONS: Mad Cow Disease, Fatal Familial Insomnia and Cannibalism

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Published on Sep 25, 2023
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What are prions? Prions are the pathogen responsible for spongiform encephalopathies, incurable diseases that affect both animals and humans: mad cow disease is certainly the most famous, but fatal familial insomnia and kuro are other examples. -- Prions and mad cow disease are terms that are often heard associated with the discussion of neurodegenerative diseases. But what exactly are prions and what is their role in the onset of mad cow disease? Prions are abnormal proteins that can cause serious problems in the brain and nervous system. These proteins can fold into an abnormal shape, turning into pathogens. Their most disturbing characteristic is the ability to convert other healthy proteins into abnormal shapes, setting off a chain reaction. Mad Cow Disease (Bovine Spongiform Encephalopathy - BSE): Mad cow disease is a neurodegenerative disease that affects livestock, especially cows. It was a major concern for the food industry and public health worldwide in the 1980s and 1990s. The disease causes devastating symptoms in animals and can be transmitted to humans through the consumption of contaminated meat. Mad cow disease is caused by prions. These prions, when present in the brain of a cow, cause healthy proteins to change into abnormal shapes, creating protein plaques that damage brain tissue. When humans consume prion-contaminated meat, they can contract a form of spongiform encephalopathy, known as variant Creutzfeldt-Jakob disease (vCJD). In addition to mad cow disease, prions are associated with other serious diseases, including fatal familial insomnia (FFI). This rare, inherited condition affects the central nervous system, causing progressively severe sleep disturbances and eventually leading to fatal complications. FFI is an example of how prions can manifest in different forms and cause devastating damage in the human body.

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