Prion Proteins and Brain Disorders
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Questions and Answers

What is the main function of the prion protein in humans and animals?

  • It plays a vital role in cell division and growth.
  • It is the primary component of the immune system.
  • It is responsible for the formation of toxic aggregations in the brain.
  • Its normal role is not yet understood. (correct)
  • What is the main difference between prions and viruses/bacteria in causing diseases?

  • Prions are not made up of DNA, unlike viruses and bacteria. (correct)
  • Prions only affect the brain, while viruses and bacteria target other organs.
  • Prions are much smaller than viruses and bacteria.
  • Prions are more easily transmitted than viruses and bacteria.
  • How do scientists theorize that HIV jumped from chimpanzees to humans?

  • Through accidental laboratory exposure.
  • Through exposure to contaminated blood.
  • Through genetic mutations that allowed the virus to adapt to humans. (correct)
  • Through the consumption of infected chimpanzee meat.
  • What is the primary reason why scientists find it difficult to understand how prions cause different strains of diseases?

    <p>Prions are identical to normal proteins except for their shape. (A)</p> Signup and view all the answers

    What scientific theory explains the cause of mad cow disease and other transmissible spongiform encephalopathies?

    <p>The prion protein adopts a pathogenic shape causing it to build up into sticky, toxic aggregations. (A)</p> Signup and view all the answers

    Why is it difficult to induce prion diseases in mammals by injecting them with a pure solution of prion proteins?

    <p>Prion proteins need other factors from infected brain matter to become infectious. (A)</p> Signup and view all the answers

    Besides mad cow disease, which other ailments could potentially be explained by researching prions?

    <p>Alzheimer's disease and Type II diabetes. (C)</p> Signup and view all the answers

    What was Stanley Prusiner's contribution to the field of prion research?

    <p>He discovered the prion protein. (B)</p> Signup and view all the answers

    What is the significance of the research conducted by Jonathan Weissman and Peter Chien?

    <p>They created a chimeric protein that could overcome the species barrier and infect both Saccharomyces cerevisiae and Candida albicans. (B)</p> Signup and view all the answers

    Why was the research on yeast particularly important for understanding prion diseases?

    <p>Yeast prion diseases exhibit similar characteristics to those found in mammals, like species-specific resistance. (D)</p> Signup and view all the answers

    Which of the following statements accurately describes the 'protein-only' hypothesis?

    <p>The 'protein-only' hypothesis states that prion diseases are caused solely by the misfolding of a single protein. (B)</p> Signup and view all the answers

    In the context of the provided text, what does the term 'promiscuous' refer to?

    <p>The ability of the chimeric protein to infect multiple species of yeast, even if they were not naturally susceptible. (D)</p> Signup and view all the answers

    What is the main implication of the research by Dr. Weissman and his team in relation to the spread of prion diseases?

    <p>They showed that prion diseases can spread between species through the transmission of misfolded prion proteins. (A)</p> Signup and view all the answers

    What is the main reason why scientists are still unsure about the exact cause of certain prion diseases?

    <p>The possibility of other biological agents contributing to the disease, besides the misfolded prion protein, remains a concern for researchers. (A)</p> Signup and view all the answers

    What is the role of the Sup35 protein in yeast?

    <p>The Sup35 protein plays a crucial role in the yeast's cellular machinery; its misfolding disrupts normal function. (D)</p> Signup and view all the answers

    What is the significance of the observation that mice are resistant to infection by brain matter from infected hamsters?

    <p>This suggests that prion diseases are species-specific and do not easily cross species barriers. (A)</p> Signup and view all the answers

    Flashcards

    Prion Theory

    A hypothesis that prions, misfolded proteins, cause infectious diseases.

    Psi-plus

    A condition in baker's yeast where misfolded Sup35 protein disrupts function.

    Chimaeric Protein

    A protein combining parts from different prion genes to study infections across species.

    Promiscuous Protein

    A protein capable of folding into multiple infectious shapes, bridging species barriers.

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    Creutzfeldt-Jakob Disease

    A degenerative brain disease in humans linked to prion infection, often from cattle.

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    Saccharomyces cerevisiae

    The scientific name for baker's yeast, studied in prion research.

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    Mammalian Prion Resistance

    Refers to certain mammals being resistant to prion infections from other species.

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    Protein-Only Hypothesis

    The theory that prions are solely proteins causing diseases, without other agents.

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    Prion

    A misshapen protein that causes brain diseases.

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    Mad Cow Disease

    A brain disorder caused by prions in cattle.

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    Transmissible Spongiform Encephalopathies

    A group of diseases caused by prions that affect the brain.

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    Stanley Prusiner

    Nobel Prize-winning neurologist who discovered prions.

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    Pathogenic Shape

    The distorted shape of prion proteins that leads to disease.

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    Strain Variation

    Different disease presentations caused by the same prion.

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    Prion Transmission

    The process by which prions spread between species.

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    Prion Protein Function

    Normal roles of prion proteins in animals are unknown.

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    Study Notes

    Prion Proteins and Brain Disorders

    • Prions are misshapen proteins linked to brain-destroying disorders like mad-cow disease.
    • They can spread between species.
    • The exact origins and infectious mechanisms are still unclear.
    • Stanley Prusiner won a Nobel Prize in 1997 for his discovery of prions.

    Prion Hypothesis and Common Diseases

    • The prion hypothesis suggests misfolded prion proteins cause diseases like mad cow by forming aggregates.
    • These diseases include bovine spongiform encephalopathy, Alzheimer's, and Type II diabetes.
    • Prion proteins are found naturally in humans and animals.
    • The specific role of prion proteins in normal body functions is not known.
    • Two mysteries of the hypothesis are the crossing of species and causation of multiple strains.

    Prion Protein Mutations and Transmission

    • Proteins, unlike viruses or bacteria, don't contain DNA and are not able to mutate and transmit genetic changes.
    • However, random mutations in prion proteins can cause different strains of disease, like different strains of the common cold.
    • Prions have the same amino acid sequence as normal proteins, but their shape differs. This shape difference is what allows for differing disease strains.

    Yeast Research and Prion Transmission

    • Yeast research is shedding light on prion transmission between species.
    • Researchers successfully created a chimaera protein from yeast, capable of bridging the species barrier.
    • This reveals that the process of prion transmission may be driven by the different protein shapes.
    • Prion diseases in mammals are difficult to create in lab settings utilizing pure prion proteins. Often, infected animal brain matter is used to create a disease.

    Prion-Only Hypothesis and Protein Buildup

    • The "protein-only" hypothesis suggests nearly any protein can potentially create toxic buildups under specific conditions.
    • A variety of proteins, including the muscle protein myoglobin, can exhibit similar prion-like behavior under specific conditions.
    • Evolution might favor proteins less prone to such transformations, as the risk of buildup of misshapen proteins increases with aging, potentially contributing to age-related diseases.

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    Secret Behind Mad-Cow PDF

    Description

    Explore the fascinating world of prion proteins and their link to neurodegenerative diseases such as mad cow disease and Alzheimer’s. This quiz sheds light on the prion hypothesis, transmission mechanisms, and the implications of mutations in these unique proteins. Test your understanding of how prions operate and the mysteries that still surround them.

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