Podcast
Questions and Answers
What is the primary cause of Scrapie in sheep?
What is the primary cause of Scrapie in sheep?
How can Scrapie be transmitted among sheep?
How can Scrapie be transmitted among sheep?
What is the consequence of prion protein misfolding in animals infected with Scrapie?
What is the consequence of prion protein misfolding in animals infected with Scrapie?
What complicates the decontamination process of premises infected with Scrapie?
What complicates the decontamination process of premises infected with Scrapie?
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How long can infected animals typically remain asymptomatic before showing signs of Scrapie?
How long can infected animals typically remain asymptomatic before showing signs of Scrapie?
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What happens to the normal function of proteins when prion proteins misfold?
What happens to the normal function of proteins when prion proteins misfold?
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What is a key characteristic of the clinical progression of Scrapie?
What is a key characteristic of the clinical progression of Scrapie?
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Which animals are primarily affected by Scrapie?
Which animals are primarily affected by Scrapie?
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What is one of the challenges related to managing an infected flock?
What is one of the challenges related to managing an infected flock?
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What is a potential source of prion contamination in meat products?
What is a potential source of prion contamination in meat products?
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What is the primary cause of Sporadic CJD?
What is the primary cause of Sporadic CJD?
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Which prion disease is primarily associated with cannibalistic practices?
Which prion disease is primarily associated with cannibalistic practices?
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What is the significance of the prion protein genes in CJD and vCJD?
What is the significance of the prion protein genes in CJD and vCJD?
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What symptom indicates that the body may be shutting down in prion disease progression?
What symptom indicates that the body may be shutting down in prion disease progression?
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How long can the incubation period be for prion diseases like CJD and vCJD?
How long can the incubation period be for prion diseases like CJD and vCJD?
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Which of the following practices significantly contributed to the spread of vCJD in the UK?
Which of the following practices significantly contributed to the spread of vCJD in the UK?
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What is a common characteristic of prion diseases?
What is a common characteristic of prion diseases?
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During what time frame was kuru reported even after its associated practices ceased?
During what time frame was kuru reported even after its associated practices ceased?
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What environmental sources can harbor scrapie prions after infected sheep are removed?
What environmental sources can harbor scrapie prions after infected sheep are removed?
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What is a distinguishing feature of inherited prion disease?
What is a distinguishing feature of inherited prion disease?
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What is the primary cause of prion diseases?
What is the primary cause of prion diseases?
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Which of the following diseases is also known as mad cow disease?
Which of the following diseases is also known as mad cow disease?
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What effect do prion diseases have on the brain?
What effect do prion diseases have on the brain?
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Why are misfolded prion proteins significant in terms of treatment?
Why are misfolded prion proteins significant in terms of treatment?
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Variant Creutzfeldt-Jakob Disease (vCJD) is primarily associated with which of the following?
Variant Creutzfeldt-Jakob Disease (vCJD) is primarily associated with which of the following?
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What is the main characteristic of prion proteins that makes them dangerous?
What is the main characteristic of prion proteins that makes them dangerous?
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Which layer of the brain is primarily affected by prion diseases?
Which layer of the brain is primarily affected by prion diseases?
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The term 'transmissible spongiform encephalopathies' refers to diseases that are primarily what?
The term 'transmissible spongiform encephalopathies' refers to diseases that are primarily what?
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What is a common misconception regarding prion diseases and their treatment?
What is a common misconception regarding prion diseases and their treatment?
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Which human condition is specifically linked to the consumption of infected beef and resembles prion diseases?
Which human condition is specifically linked to the consumption of infected beef and resembles prion diseases?
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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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Description
This quiz explores prion diseases, including their causes, effects on humans and animals, and specific conditions like Mad Cow Disease and Scrapie. Understand the role of misfolded prion proteins and their long-lasting impacts on health.
