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20 Cards in this Set
- Front
- Back
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What is a Prion
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Not caused by a virus, bac, fung, or parasite. Caused by a vairation in the conformation of a normal cellular protein found in the CNS
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How can it start
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Introduction of foreign homolog with aberrant conformation
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From whom is it contracted from ie H or An
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humans and animals
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Other ways it can start
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Genetic mutation or by chance
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A Prion is.....
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Proteinaceous infectious particles
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Has viral nucleic acid been found associated with a Prion
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NO
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Prions are resistant to......
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anything harmful to DNA such as UV, formaldehyde, ionizing radiation
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Sensitive to.....
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protein denaturing agents, phenol, urea, guanidine, HCL, NaOH
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Does it cause inflammation
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NO, not really. No AB response
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what is the best studied one
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The one that causes scrapie in sheep
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Proposed Molecular Disease Process
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PrPsc, protease resistant prion protein causes molecular switch of cellular protein
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PrPc to the prion conformation
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Altered protein cannot be degraded by cellular proteases and accumulates in cytoplasm and extracellular areas of cells in the brain
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Kuru
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New guinea-seems to be transmitted by brain of the dead during preparation for ritual cannibalism
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GSS
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prion protein mutation of P102L and others
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CJD
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Especially found in jewish Libyans, but there is some all over the world. It may be sporadic, passed accidentally via human tissue transplantation or hereditary
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FFI
Fatal Familial Insomnia |
atrophy of hte anterior ventral and mediodorsal thalamic nuclei
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vCJD
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derived from consumption of meat from cows suffering from Bovine Spongiform Encephalopathy
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CJD
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genetic form arises from mutation in PRP.
Transmission in Japan by grafts of dura mater during neurosurgery. In the case the age on onset is somewhat less CJD can also arise spontaneously in the population and has a rate of 3.1/million |
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Scrapie
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Sheep lose wool
Fatal neuropathy |
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Bovine spongiform encelphathy BSE
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progressive disease in cattle resulting from tremors
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