Lecture 12

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Questions and Answers

What is the primary cause of Scrapie in sheep?

  • Viral infection
  • Prion protein misfolding (correct)
  • Bacterial infection
  • Fungal infection

How can Scrapie be transmitted among sheep?

  • Through contaminated food only
  • Exclusively through shared water sources
  • Only from mother to offspring
  • Via environmental contamination or direct contact (correct)

What is the consequence of prion protein misfolding in animals infected with Scrapie?

  • Enhanced immune response
  • Immediate recovery from illness
  • Progressive neurodegeneration (correct)
  • Improved neurological function

What complicates the decontamination process of premises infected with Scrapie?

<p>The prion's ability to survive in harsh conditions (C)</p> Signup and view all the answers

How long can infected animals typically remain asymptomatic before showing signs of Scrapie?

<p>Months to years (A)</p> Signup and view all the answers

What happens to the normal function of proteins when prion proteins misfold?

<p>They lose their functionality (B)</p> Signup and view all the answers

What is a key characteristic of the clinical progression of Scrapie?

<p>Symptoms are progressive and fatal (A)</p> Signup and view all the answers

Which animals are primarily affected by Scrapie?

<p>Sheep and goats (A)</p> Signup and view all the answers

What is one of the challenges related to managing an infected flock?

<p>Long incubation period before symptoms appear (C)</p> Signup and view all the answers

What is a potential source of prion contamination in meat products?

<p>Untreated or contaminated meat from infected animals (C)</p> Signup and view all the answers

What is the primary cause of Sporadic CJD?

<p>Unknown etiology (B)</p> Signup and view all the answers

Which prion disease is primarily associated with cannibalistic practices?

<p>Kuru (A)</p> Signup and view all the answers

What is the significance of the prion protein genes in CJD and vCJD?

<p>They exhibit specific genetic profiles. (D)</p> Signup and view all the answers

What symptom indicates that the body may be shutting down in prion disease progression?

<p>Severe coordination issues (C)</p> Signup and view all the answers

How long can the incubation period be for prion diseases like CJD and vCJD?

<p>Years (A)</p> Signup and view all the answers

Which of the following practices significantly contributed to the spread of vCJD in the UK?

<p>Eating contaminated meat and meat products (D)</p> Signup and view all the answers

What is a common characteristic of prion diseases?

<p>They are invariably fatal. (A)</p> Signup and view all the answers

During what time frame was kuru reported even after its associated practices ceased?

<p>For several decades (A)</p> Signup and view all the answers

What environmental sources can harbor scrapie prions after infected sheep are removed?

<p>Water troughs and feed (B)</p> Signup and view all the answers

What is a distinguishing feature of inherited prion disease?

<p>It runs in families due to specific genetic traits. (A)</p> Signup and view all the answers

What is the primary cause of prion diseases?

<p>A misfolded prion protein (B)</p> Signup and view all the answers

Which of the following diseases is also known as mad cow disease?

<p>Bovine spongiform encephalopathy (D)</p> Signup and view all the answers

What effect do prion diseases have on the brain?

<p>They lead to the degeneration of brain tissue (D)</p> Signup and view all the answers

Why are misfolded prion proteins significant in terms of treatment?

<p>They are resistant to heat and protease treatments (C)</p> Signup and view all the answers

Variant Creutzfeldt-Jakob Disease (vCJD) is primarily associated with which of the following?

<p>Contaminated beef products (D)</p> Signup and view all the answers

What is the main characteristic of prion proteins that makes them dangerous?

<p>Their ability to misfold and induce other proteins to misfold (D)</p> Signup and view all the answers

Which layer of the brain is primarily affected by prion diseases?

<p>Inner layer of the brain (C)</p> Signup and view all the answers

The term 'transmissible spongiform encephalopathies' refers to diseases that are primarily what?

<p>Characterized by abnormal protein accumulation (D)</p> Signup and view all the answers

What is a common misconception regarding prion diseases and their treatment?

<p>They are easily treatable with common medications (C)</p> Signup and view all the answers

Which human condition is specifically linked to the consumption of infected beef and resembles prion diseases?

<p>Variant Creutzfeldt-Jakob disease (B)</p> Signup and view all the answers

Flashcards

What is a prion?

A prion is an infectious agent that can cause disease by altering the shape of normal proteins in the brain.

How do prions cause disease?

Misfolded prion proteins convert normal proteins into misfolded versions, causing them to clump together and damage brain cells.

Why is it hard to kill prions?

Prions are resistant to heat and other methods often used to kill bacteria and viruses.

What is Scrapie?

Scrapie is a neurodegenerative disease caused by a prion, primarily affecting sheep and goats.

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How does Scrapie spread?

Scrapie can be transmitted between animals through direct contact or contaminated environments, making it challenging to control outbreaks.

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What are the symptoms of Scrapie?

Animals infected with Scrapie can remain asymptomatic for several years before developing progressive and fatal neurological symptoms.

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Is cooked contaminated meat safe?

Heat can kill most bacteria and viruses, but not prions. Therefore, consuming cooked contaminated meat can still pose a risk.

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Can prions be inherited?

Inherited mutations in a gene can cause prions to misfold, leading to the development of prion diseases.

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What does a prion disease do to the brain?

Prion diseases can affect humans and animals, causing various neurodegenerative disorders.

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Is there a cure for prion diseases?

Once prions are in the body, there is no cure for prion diseases. Treatment focuses on managing symptoms.

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Prion

A type of protein that can cause fatal brain diseases. Prions are unique because they are misfolded proteins that can cause other normal proteins to misfold, leading to a chain reaction that damages brain cells.

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Scrapie

A fatal brain disease in sheep caused by prions. It's characterized by coordination problems, loss of balance, and eventual death.

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CJD (Creutzfeldt-Jakob Disease)

A fatal brain disease in humans caused by prions. It's the most common type of human prion disease and can occur sporadically or be inherited.

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vCJD (Variant Creutzfeldt-Jakob Disease)

A rare variant of CJD that emerged in the 1990s and is linked to the consumption of contaminated beef.

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Kuru

A fatal brain disease in humans caused by prions that was once common in New Guinea due to a ritualistic practice of eating deceased relatives' brains.

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Incubation period

The time period between exposure to a prion and the development of disease symptoms.

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BSE (Bovine Spongiform Encephalopathy)

A fatal brain disease in cattle caused by prions. It's known for causing a sponge-like appearance in the brain.

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Prion replication

The process by which prions cause normal proteins to misfold, leading to the formation of more prions and brain damage.

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Pores in the brain

The holes and gaps that develop in brain tissue due to prion-induced damage. These pores contribute to the sponge-like appearance of the brain.

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Symptoms of Prion Disease

The characteristic signs and symptoms of prion diseases, including coordination problems, loss of balance, and eventual death.

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What are prion diseases?

Prion diseases are a group of fatal neurodegenerative disorders caused by misfolded proteins called prions.

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What are prions?

Prions are misfolded versions of a normal protein found in the brain, primarily neurons.

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How do prion diseases affect the brain?

Prion diseases are characterized by the accumulation of misfolded prion proteins in the brain, leading to brain damage and ultimately death.

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Why are prion diseases difficult to treat?

The misfolded prion protein is highly resistant to degradation by heat and proteases.

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What is Mad Cow Disease?

Bovine Spongiform Encephalopathy (BSE), commonly known as Mad Cow Disease, is a prion disease affecting cattle.

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How can humans get infected with prion diseases?

Mad Cow Disease can be transmitted to humans through consuming contaminated beef, leading to a variant of Creutzfeldt-Jakob Disease (vCJD).

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What is variant Creutzfeldt-Jakob Disease (vCJD)?

Variant Creutzfeldt-Jakob Disease (vCJD) is a human prion disease linked to consuming contaminated beef. It's a human version of Mad Cow Disease.

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Can prion diseases be inherited?

Prion diseases can be inherited, meaning a genetic predisposition exists. This means some individuals are more likely to develop prion diseases due to their genes.

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What are the effects of prion diseases on humans?

Prion diseases cause severe neurological damage leading to dementia, memory loss, behavioral changes, and ultimately death.

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Study Notes

Prion Diseases

  • Prion diseases, also known as Transmissible Spongiform Encephalopathies (TSEs), are caused by an infectious misfolded prion protein.
  • This misfolded protein is abundant in neurons.
  • The misfolded prion protein is resistant to heat and protease treatments.
  • Mad cow disease (bovine spongiform encephalopathy, BSE) and variant Creutzfeldt-Jakob disease (vCJD) are examples of prion diseases found in humans.
  • Misfolded prion proteins transform normal proteins, causing them to misfold and aggregate in the brain's neurons.

Learning Objectives

  • Students will be able to describe prion diseases.
  • Students will be able to explain impacts of prion diseases on humans.

Scrapie

  • Scrapie is a neurodegenerative disease caused by a prion.
  • It primarily affects sheep and sometimes goats.
  • Infected animals may not show symptoms for years before progressing to a fatal illness.
  • Clinical signs are progressive and invariably fatal once they develop.
  • Scrapie is transmissible between animals, either directly or environmentally.
  • Contaminated environments, like feed and water troughs, can spread scrapie. (20 days after infected sheep removal, risk remains)

Prion Diseases in Humans

  • Sporadic CJD (Creutzfeldt-Jakob Disease) is the most common prion disease in humans, affecting approximately 1.7 million people worldwide annually.
  • Inherited prion diseases are genetically influenced, with specific genetic profiles of the prion protein gene.
  • Acquired prion diseases occur from ingesting prions, such as Kuru in New Guinea (practice of eating brain tissues stopped in 1960, however, cases appeared many years after).
  • Variant CJD (vCJD) emerged in the UK around 1996. The link between vCJD and consuming contaminated beef (BSE) is significant.

Signs and Symptoms

  • Prion diseases have very long incubation periods (years).
  • Clinical outcomes often include dementia, loss of coordination, deterioration, and death.
  • Some prion diseases can arise spontaneously, while others are inherited.
  • Prion diseases are acquired when the prion enters the digestive tract, replicates within the lymphatic system (spleen, lymph nodes, Peyer's patches), and then travels to the central nervous system (CNS).

Treatment

  • Prion diseases cannot be cured.
  • Treatments focus on alleviating symptoms and ensuring patient safety.
  • Research is underway for anti-prion compounds to block the misfolding process (PrPC to PrPSc).
  • Prevention strategies, such as food safety precautions, are essential.

General Observations

  • Prions cause brain damage characterized by pore formation, resulting in spongy appearance under microscopy. This is visible in images of Kuru, Scrapie, and CJD brains.

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