TSEs are currently thought to be due to the accumulation of abnormal clumps of protein in the neurons of the brain. As the protein accumulates within the neuron, it crowds out normal structures and eventually causes the neuron to die. When the neuron dies, it leaves a characteristic hole in the histologic structure of the brain, and when enough neurons die the animal develops neurologic symptoms and eventually dies.
Examples of TSEs in animals include bovine spongiform encephalopathy (BSE, also called mad cow disease), scrapie (a disease in sheep that has been around for at least 200 years), and chronic wasting disease (CWD, affecting elk, moose, deer and other cervids in North America). Of these, only BSE has been shown to be zoonotic (infectious to humans).
The transmissible spongiform encephalopathies (BSE, CWD, kuru, etc.) are not viruses - they are infectious mis-folded proteins called prions.
A prion is a misfolded protein that is considered an infectious agent because they cause properly folded proteins to convert into the misfolded, prion form. In humans, prions cause Creutzfeldt-Jakob disease. In cattle, prions cause mad-cow disease.
Transmissible Spongiform Encephalopathy: Scrapie in sheep and Mad Cow disease (or Bovine Spongiform Encephalopathy)
Spongiform encephalopathies are thought to be caused by abnormal prion proteins that trigger a cascade of events leading to neuronal death, resulting in sponge-like holes in the brain tissue. These diseases include Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob disease, and bovine spongiform encephalopathy (mad cow disease).
There are no transmissible spongiform encephalopathies that cause cannibalism. However, there was a TSE in humans that was transmitted through ritual cannibalism. This was kuru, a disease identified in indigenous people in the South Pacific. Part of the culture was for the survivors to consume the bodies of those who had died, so that the deceased could continue in some fashion within those who had survived. However, once the link between ritual cannibalism and death from kuru was established, the people abandoned the practice and kuru has been effectively eradicated from this population.
Transmissible Spongiform Encephalopathy.
PrionsA prion is an infectious protein that is misfolded. These proteins can aggregate in the brain and other neural tissue, forming amyloids. Diseases associated with prions include bovine spongiform encephalopathy (mad cow disease), scrapie, kuru, chronic wasting disease, and Creutzfeldt-Jakob disease. Prions are still poorly understood by researchers, and prion diseases (transmissible spongiform encephalopathies) remain untreatable.
Consuming cooked cow brains may pose health risks due to concerns about transmissible spongiform encephalopathies, such as Creutzfeldt-Jakob disease. These diseases are serious and potentially fatal. It is generally recommended to avoid consuming cow brains to reduce the risk of these prion-related illnesses.
There are a range of transmissible spongiform ensephalopathies that affect the brain or other neural tissue.Bovine spongiform encephalopathy (mad cow disease)Creutzfeldt-Jakob diseaseChronic wasting diseaseScrapieFeline spongiform encephalopathyKuruFatal familial insomniaGerstmann-Sträussler-Scheinker syndrome
Transmissible Spongiform Encephalopathy: Scrapie in sheep and Mad Cow disease (or Bovine Spongiform Encephalopathy), cows can get TB and sheep can get foot and mouth
Prions are proteins that can cause misfolding of other proteins in the brain, leading to neurodegenerative diseases like Creutzfeldt-Jakob disease in humans. Due to their ability to self-propagate and resist normal sterilization techniques, prions pose a significant risk of spreading through contaminated tissues or medical instruments. Transmissible spongiform encephalopathies caused by prions are currently incurable and fatal.
Prions are misfolded proteins that can induce abnormal folding of normal proteins in the brain, leading to neurodegenerative diseases. They are primarily associated with various transmissible spongiform encephalopathies (TSEs), such as Creutzfeldt-Jakob disease in humans, mad cow disease in cattle, and scrapie in sheep. Prions can be found in infected tissues, particularly in the brain and nervous system of affected animals and humans. They are notable for their resistance to conventional methods of sterilization and disinfection.