BIOA01H3 Lecture Notes - Lecture 39: Bovine Spongiform Encephalopathy, Transmissible Spongiform Encephalopathy, ChoreaPremium

Biological Sciences
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Aarthi Ashok, Shelley Brunt, Mark Fitzpatrick

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BIOA01 Lecture 36: Prions
- Discovery of Prions
o 1: South Forray tribe in Papa New Guinea came down with Kunu disease in the
Women and children were mostly affected
They suffered involuntary muscle movements, muscle wasting
and eventually death
Given 3 to 6 months to live
Daniel Gajdusck discovered that the tribe had cannibalistic practises: it
was mainly the women and children that took part in it
The people that took part in these practises ended up with the
Their brains looked like sponges compared to normal people
o Neuron death left gaps resulting in smaller brain size
o 2: Farmers in UK noticed similar sponge brain symptoms in their cows
Cows also had involuntary muscle movement and muscle wasting
Farmers connected this to a prior disease seen in sheep
Disease was called scrappy in sheep
o Involved the same symptoms and the same spongy brain
Farmers admitted to feeding the dead sheep that died from
scrappy to the cows
o Cows thus developed BSE
o BSE: bovine spongiform encephalopathy
o Therefore the spongiform encephalopathy jumped from
the sheep to the cows
TSE: transmissible spongiform encephalopathy
Could eventually reach humans
o Stanley Phrismor: proposed that proteins were pathogens
Looked at the relative contributions of DNA and proteins to the spread of
these diseases
1: took infected brain tissue and added agents that cut DNA
o Then put the tissue into a healthy chimp
o If DNA was responsible for transmitting the disease,
destroying the DNA would have resulted in the chimp
being unaffected
o Results
Chimp got the disease and died
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