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34 Cards in this Set
- Front
- Back
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Define prions |
Small proteinaceous infectious particles that resist inactivation by procedures that modify viruses and nucleic acids; resist inactivation--high heat, acid; Prions are misfolded proteins that cause like proteins to also midfold |
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Define prion diseases |
high levels of misfolded prion proteins that are transmissible |
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What are amyloid diseases? |
amyloid fibers found in the brain that are non-transmissible |
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About how many people in the U.S. does Alzheimer's affect? |
4.5 million people |
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About how many people in the U.S. does Parkinson's affect? |
500,000 people
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About how many people in the U.S. does Huntington's affect? |
30,000 people
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What is CJD? |
It is a neurological disease which occurs sporadically in humans at a ratio of 1 per 1 million people; Estimated that 1 per 10,000 people have CJD at the time of death (estimate could be inaccurate as CJD could be mistaken for similar neurological diseases) |
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What are two types of CJD? |
PrPc and PrPsc proteins |
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How is CJD caused? |
It is naturally occurring due to a mutation in a gene that encodes the PrPc neural protein |
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How is vCJD caused? |
Consuming meat from cattle with BSE who are infected with the PrPsc protein |
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PrPsc is only transmissible from one human to another via ________ |
Corneal transplants, brain surgery w/ contaminated instruments, and contaminated w/ brain probes |
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Post-mortem examination of the brain and neural tissue and cells show __________ |
amyloid (starch-like) protein deposits--plaques between cells, non-inflammatory lesions, strange formations of neurons, vacuoles--large membrane bound inclusion bodies |
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What age does naturally occurring CJD affect humans? |
50-60 years old |
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vCJD from transmissible prions can affect people as early as |
14 years old |
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What are CJD disease manifestations? |
shaking, loss of motor control, dementia-- memory loss, paralysis, pneumonia, death--a few months to 1.5 years after first symptoms |
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What are the protein properties? |
Prions are misfolded proteins, aggregate in amyloids, infectious, propagate into other cells. |
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Where are PrPc usually found? |
Surface of brain cells |
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In some individuals a rare mutation on chromosome 20 can cause _______ |
PrPc to misfold into PrPsc |
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After awhile vCJD turns into _________ |
Mad Cow Disease |
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How does a human develop PrPsc? |
The misfolded protein enters the human's nerve cells and travels to the brain tissue. |
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What is the only way PrPsc is tranmissible to humans? |
from cows with BSE |
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Cattle can also transmit disease to |
domestic cats, sheep, and pigs |
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What is CWD (Chronic Wasting Disease)? |
In elk and muledeer--a disease similar to BSE in cattle; not documented as transmissible in humans |
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Is PrPsc transmissible through milk and milk products? |
No, it is not transmissible through milk and milk products |
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What occurs naturally in cows at a low rate as CJD does in humans? |
Hereditary BSE |
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PrPsc can be found concentrated in the following areas in cows _________. |
Brain, Spinal Cord, Retina (Eye), Distal Ileum (Small Intestines), Neurons near the Backbone, Bone Marrow, Lymphatic Tissue |
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Scrapies are found in the ________ |
B-Sheets |
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What does BSE stand for? |
Bovine Spongiform Encephalepathy |
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What does Bovine Spongiform Encephalepathy mean? |
Spongiform means the brain has a sponge-like appearance; Encephalepathy means pathology of the brain |
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Kuru happens in who? |
Humans |
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Creutzfeldt-Jakob disease happens in who? |
Humans |
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How does someone get vCJD? |
Humans-- via medical procedures and from consuming beef from a cow with BSE |
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PrPc is ________ |
Alpha Helices |
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PrPsc is ______ |
Beta-Sheets |
