Prions Flashcards
what does Kuru mean
trembling from fear and cold
what is the clinical course for Kuru
prolonged headache and minor loss of coordination. symptoms rapidly progressed and by 4 months victims unable to walk requiring constant care. Speech became disturbed and completely disappeared. Victims lost ability to swallow. Death occurred within 1 year of onset of symptoms
When did Kuru first appear
~1900
Kuru was a disease primarily of who
women and children
What was the name of the tribe of people who experienced Kuru
Fore in New Guinea
What seemed to be the most reasonable cause of Kuru
Cannibalism
Why was cannibalism practiced in the New Guinea tribe
Was a religious ceremony in which the women would eat the flesh of the dead, mainly the brain.
How did the New Guinea religious ceremony start
The men ate all the food and the women were starving, so they begin to eat the dead to get protein and disguised it as a religious ceremony
What characteristics about the brains taken resembled another disease
loss of neurons (spongiform encephalopathy) and activation of astrocytes
Which disease did the Kuru brains resemble
Creutzfeldt-Jakob disease
What is Creutzfeldt-Jakob disease also known as and why
Spongiform encephalopathy due to the gaping holes where the neurons had once been
What test animals was chosen to be inoculated with the Kuru brain
Rhesus monkeys and Chimpanzees
How many people were dying of Kuru in the Fore tribe
200 persons each year (1% of tribe)
What was the ratio of men to women
3:1
The women used ____ to prepare the tissues and packed the brain in small ____ to be steamed over an open fire
Bamboo blades; bamboo tubes
When NIH experiments and duplicates the preparation conditions what did they find
10 million infectious agents per gram of steamed brain
What impact on medicine, neurology, and infectious disease did Kuru have
opened new area of slow virus diseases, opened new area of transmissible spongiform encephalopathy, and lead to identification of new infectious agent ,the prion
Viruses are considered ____ agents and prions are considered ____ agents
Conventional; unconventional
Subacute Sclerosing Panencephalitis is caused by what
a measles virus with defective Matrix protein
What is the incubation period of SSP
7-10 days
What is the clinical progression of SSP
Progressive dementia , ataxia, and myoclonus. Death usually occurs within 1-3 years
What are some slow virus diseases found in animals
Scrape in sheep; Transmissible mink encephalopathy; Bovine spongiform encephalopathy
What are some slow virus diseases found in humans
Creutzfeldt-Jakob disease; Variant Creutzfeldt-Jakob disease (mad cow); Kuru (but kuru is extinct)
What is the prion hypothesis
the prion is a small proteinacious infectious particle which is resistant to inactivation by most procedures that modify nucleic acids
