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study guide 3
Prions
| Question | Answer |
|---|---|
| How are infectious prion particles acquired? | through the diet, via a mutation in the gene which codes for the prion protein In humans |
| In humans, what is the name of the disease when the prion is acquired from one’s diet? | variant CJD |
| Why is it so difficult for scientists to determine how much prion protein it takes to cause disease? | once the infectious prion enters the brain, it will trick normal prions into changing shape and causing disease |
| If an animal has a mutation in the gene which codes for the prion protein, and the resulting prions are infectious and kill the animal, there is no danger to someone else unless the animal is used for feed for other animals | true |
| Match the following disease with the appropriate description: prion disease in elk | chronic wasting disease |
| Match the following disease with the appropriate description: prion disease in humans | CJD (Creutzfeld-Jacob Disease |
| Match the following disease with the appropriate description: prion disease in deer | chronic wasting disease |
| Prions target and kill: | Neurons |
| A prion is a: | Protozoan |
| The danger to society is not the sporadic CJD but variant CJD. Sporadic CJD will only affect the person in whom the mutation took place. In variant CJD, the infectious prion is in the food and has the potential to infect large numbers of people. | True |
| Prions cause | Spongiform encephalopathy |
| Alzheimer’s and CJD appear to have some similarities. Both diseases cause neurological dysfunction. How do we know that they are not the same disease? | ) the protein causing Alzheimer’s is amyloid and not a prion and Alzheimer’s histology does not show the spongiform appearance seen in CJD |
| The difference between the figures above is that the secondary protein structure of the figure on the left has: | more alpha-helices |
| A tribe in Papua, New Guinea was plagued with a disease called Kuru. It was eventually determined that Kuru was a prion-mediated disease. How were members of the tribe being exposed to the infectious prion particle? | eating the remains of their dead as part of their religious practices |
| Match the following disease with the appropriate description:mad cow disease | BSE (bovine spongiform encephalopathy) |
| What is its target tissue for prion disease? | nerve cells |
| What disease do prions cause? | spongiform encephalopathy |
| What cell type in the body do prions kill and how? | Brain tissue, by forming lesions in the brain tissues |
| what is the specific name of this disease for the sheep | scrapie |
| 5.what is the specific name of this disease for the cow | bovine spongiform encephalopathy |
| 6.what is the specific name of this disease for the Deer/elk | Chronic Wasting Disease |
| what are the two different kinds of CJD | Sporadic Creutzfeld-Jacob Disease and Variant CJD |
| Sporadic Creutzfeld-Jacob Disease | produced by the individual due to mutation or inheritance and |
| Variant CJD | acquired through the diet |
| What does ‘CJD’ stand for? | Creutzfeld-Jacob Disease |
| How do animals acquire the prion? | Mutation occurs in PrP gene and animal produces PrPsc, develops disease and then dies |
| What are the symptoms associated with prion disease? | dementia, muscle incoordination; tremors, |
| What other disease has similar symptoms? | Alzheimer’s |
| know the different levels of structure of a protein and know the two kinds of secondary structure | α-helices (coils), β-pleated sheet |
| Which secondary structure is dominant in PrPsc? | β-pleated sheets |
| Which one is infectious? | PrPsc |
| What effect does the infectious form have on the normal brain protein | acts as a template to refold normal brain protein (PrPC): α-helices→ β-pleated sheets |