Prion Proteins and Brain Disorders
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Questions and Answers

Which of the following statements is TRUE regarding the prion theory?

  • It has been proven definitively.
  • It is considered a working hypothesis with considerable supporting evidence. (correct)
  • It is based solely on the observation of yeast, and cannot be extrapolated to other species.
  • It is a purely hypothetical theory with no supporting evidence.

    • What is the significance of the 'chimaeric' prion protein created by Dr. Weissman and his student?

  • It showed that the chimaeric protein only mimics the behavior of one of its parent proteins.
  • It proved that prions from one species can never infect another.
  • It demonstrated that the chimaeric protein could overcome the species barrier and infect both types of yeast. (correct)
  • It was a failure as it did not display any infectious properties.

    • What did Jonathan Weissman and Peter Chien's research on yeast help to explain?

  • How prions can be cured.
  • How prions affect the immune system.
  • The existence of prion strains and their ability to jump between species. (correct)
  • The genetic origin of all prion diseases.

    • What is 'psi-plus' in the context of yeast?

    <p>A condition where a misfolded protein disrupts the yeast's cellular machinery. (C)</p> Signup and view all the answers

    What was the unexpected result of the experiment with the chimaeric prion protein?

    <p>It exhibited 'promiscuous' behavior and infected both types of yeast. (D)</p> Signup and view all the answers

    What is the significance of the research on prions in yeast in relation to prion diseases in mammals?

    <p>It provides evidence that prions can cross species barriers, potentially explaining how mad-cow disease affected humans. (B)</p> Signup and view all the answers

    What is the underlying principle behind the 'protein-only' hypothesis in relation to prions?

    <p>Proteins can fold into multiple infectious shapes. (A)</p> Signup and view all the answers

    The research on yeast, as described in the text, suggests that prions can:

    <p>Be transferred from one species to another. (B)</p> Signup and view all the answers

    What is the main function of prion proteins?

    <p>Their normal function is not yet understood. (C)</p> Signup and view all the answers

    How do prions differ from viruses and bacteria in terms of their genetic makeup?

    <p>Prions do not contain a genetic code, while viruses and bacteria do. (A)</p> Signup and view all the answers

    What is the primary difference between prions and normal proteins?

    <p>Prions have a different shape than normal proteins. (B)</p> Signup and view all the answers

    How are different strains of prion diseases thought to be caused?

    <p>Changes in the shape of the prion protein. (D)</p> Signup and view all the answers

    What has been a major obstacle for researchers trying to study prion diseases in the lab?

    <p>The inability to replicate prion diseases by injecting pure prion proteins. (D)</p> Signup and view all the answers

    What is the connection between bovine spongiform encephalopathy (mad cow disease) and humans?

    <p>Prions from infected cattle can be transmitted to humans through the consumption of contaminated meat. (B)</p> Signup and view all the answers

    What is a possible reason why scientists believe that understanding prion diseases could shed light on other ailments, such as Alzheimer's disease and Type II diabetes?

    <p>They all involve misfolded proteins that aggregate in the body. (D)</p> Signup and view all the answers

    What is one of the biggest mysteries surrounding prion diseases?

    <p>How prions are able to cause different strains of the same disease. (D)</p> Signup and view all the answers

    Flashcards

    Prion Theory

    Hypothesis that prions, misfolded proteins, cause infectious diseases.

    Chimaeric Prion Protein

    Fusion of prion protein genes from different yeast species.

    Psi-plus

    A condition in yeast where misfolded Sup35 protein disrupts function.

    Sup35 Protein

    A protein in yeast that can misfold and form toxic clumps.

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    Strains Cross Species Barrier

    The ability of prion strains to infect different species.

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    Infectious Shapes of Proteins

    Proteins that can assume multiple forms, facilitating disease spread.

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    Protein-Only Hypothesis

    Idea that proteins alone are responsible for prion diseases.

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    Toxic Buildups

    Accumulation of misfolded proteins leading to toxic effects in cells.

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    Prion

    A misshapen protein that can cause brain disorders.

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    Mad-cow disease

    A brain ailment caused by prions in cattle.

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    Stanley Prusiner

    Neurologist who discovered prions and won the Nobel Prize.

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    Infectious cross-species

    The ability of prions to jump from one species to another.

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    Transmissible spongiform encephalopathies

    Diseases caused by prion proteins' pathogenic shape.

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    Normal prion proteins

    Proteins occurring normally in humans and animals.

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    Prion disease induction

    Prion diseases cannot be caused by injecting pure prion solutions.

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    Disease strains

    Different variations of illnesses linked to prions.

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    Study Notes

    Prion Proteins and Brain Disorders

    • Prions are misshapen proteins believed to cause mad cow disease and similar human brain ailments in humans, sheep, and other species
    • Prion origins, how they become infectious between species, and their disease mechanisms are still unclear
    • Stanley Prusiner won the Nobel Prize in 1997 for his prion discovery
    • Scientists are piecing together clues to understand bovine spongiform encephalopathy (BSE) outbreaks and their spread to humans via the food supply
    • Prion theory suggests that a prion protein shape change causes toxic aggregations
    • Prions are normally present in humans and animals
    • Prions can cross species barriers and cause different disease strains
    • Unlike other diseases, prions don't rely on genetic mutations; their shape differences cause different disease strains
    • Similar, random mutations create varied strains of diseases like the common cold
    • Chimaeric prion proteins, combining different yeast prion protein genes, have caused species bridging, showing prions can change shape, not just genetic code

    Prion Diseases in Mammals

    • Prion diseases are difficult to create in mammals due to the need for purified solutions from infected animal brains
    • Impurities in these solutions may contain other disease agents
    • Scientists are investigating the role of prion proteins and their different shapes in disease
    • Research on yeast shows that a single protein can take on various infectious shapes
    • Prion-like proteins in yeast aren't infectious across species in some cases, like the yeast Saccharomyces cerevisiae infecting Candida albicans
    • Prion proteins from one species can infect another via "chimaeric" proteins, composed of parts of different prion proteins, leading to species-bridging
    • These chimaeric proteins can shift shape across species

    Prion-Only Hypothesis and Protein Transformations

    • The "protein-only" hypothesis suggests prions are responsible for crossing species
    • Various proteins can have potentially toxic buildups under certain conditions, similar to prion behavior, even a protein like myoglobin
    • Evolution may have favored proteins less prone to these shape changes due to protein stability concerns with aging
    • Accumulation of misfolded proteins is linked to aging diseases like Alzheimer's, Parkinson's, and Type II diabetes

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    Secret Behind Mad-Cow PDF

    Description

    Explore the fascinating world of prion proteins and their role in brain disorders. This quiz covers the origins, mechanisms, and infectious nature of prions, including insights from Nobel Prize winner Stanley Prusiner. Test your knowledge on how these abnormal proteins impact human and animal health.

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