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8 Cards in this Set
- Front
- Back
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Transmissible Spongiform Encephalopathies cause problems due to
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failure to clear a protein (prion) leads to neuronal death
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what is the major distinguishing key of prion diseases
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transmissibility of a protein can lead to dz in other ppl
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what structure of prion is damaging to a host? what is importance about this form?
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B-sheet (aberrant form)
Aberrant forms are resistant to degradation, and are non-immunogenic to host |
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common pathogenesis of Creutzfeld-Jacob Disease (CJD)
another important way? |
spontaneous conversion from alpha to beta prions
passed by person to person (reusing surgical tools, eating mad cow beef) |
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man hunts a deer and wants to take it home and eat it. If he is in CO, WV or a few other places what dz does he have to worry about?
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Chronic wasting disease (CWD, deer and elk)
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all ppl who had nvCJD had changes where
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homozygous methionine at codon 129
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Rapidly progressive dementia and myoclonus. Death within year of onset. Due to an infective protein
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Creutzfeld-Jakob Disease
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what 2 ways can you distinguish mad cow (nvCJD) from sporadic CJD?
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ncCJD: “florid” plaque
other finding is “Pulvinar sign” in MRI |
