Term
| On what chromosome is the gene that encodes prion protein |
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Definition
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Term
| What are the symptoms of scrapie? |
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Definition
| ataxia, tremor, weakness, and excessive thirst; also an intense itch that causes sheep to scrape off their wool |
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Term
| What are the characteristics of Kuru? |
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Definition
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Term
| What is the incidence of sporadic CJD worldwide? |
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Definition
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Term
| What histologic characteristics are seen all TSE diseases? |
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Definition
- spongiform change
- loss of neurons
- reactive astrocytosis/gliosis
- amyloid plaque deposition
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Term
| What are some changes in the biochemical properties of abnormal prion protein? |
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Definition
| decreased detergent solubility and increased resistance to enzymatic degradation |
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Term
| What are four ways prion disease can be acquired? |
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Definition
- use of improperly sterilized neurosurgical instruments
- corneal transplants or dural grafts
- administration of contaminated growth hormone
- consumption of food products contaminated with bovine spongiform encephalopathy (vCJD)
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Term
| What are some observed indications of prion strain diversity? |
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Definition
- different incubation periods
- different cerebral distribution
- different post-proteinase K Western banding pattern
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Term
| What are some common symptoms of CJD? |
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Definition
- changes in behavior, altered emotional responses, decline in intellect
- altered motor and cerebellar function
- myoclonic contractions
- sharp wave complexes on EEG
- invariably fatal
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Term
| What are two inherited TSEs? |
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Definition
Gerstmann-Straussler-Scheinker Disease
Fatal Familial Insomina |
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Term
| What are the two most common mutations leading to GSS? |
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Definition
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Term
| Which prion disease is characterized by ataxia, dysarthria, and nystagmus? |
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Definition
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Term
| What mutation in codon 129 causes fatal familial insomnia and what are the symptoms? |
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Definition
| D178N; insomnia, sympathetic over-activity, endocrine disturbances |
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Term
| What is a definitive way to diagnosis TSE? |
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Definition
| proteinase K resistant form of prion protein in brain tissue |
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Term
| What sort of polymorphisms are seen at codon 129 of PrP? |
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Definition
- homozygous valine -> iatrogenic CJD
- homozygous methionine -> FFI
- all vCJD patients -> homozygous methionine
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Term
| What is the mean age of death in patients with vCJD? |
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Definition
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Term
What is chronic wasting disease? |
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Definition
- affects mule, deer and elk
- not known to naturally infect other species of wildlife
- no treatment; fatal
- infect both free-ranging and captive animals
- potential public health threat
- Presently there is no evidence that humans can contract a form of spongiform encephalopathy from eating CWD positive wild deer and elk meat.
- can be transmitted by deer excreta or decomposed deer carcasses
- of all the mammalian prion diseases it is the most efficiently transmitted
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