What are Plions? Mad Cow Disease

They are called prions. They are not easily treatable because they are proteins that are identical in sequence to an already existing protein in the body, but are a different shape. Most immune responses target something that is different than what exists in the body.
 
hmmm

why is it they are hard to get rid of? because they have tried incinerating cows and in the ashes there still lie prions
 
Hmm, I haven't heard that...do you have a link, or a source of any kind? The only thing I can think of would be incomplete combustion, but that would depend on method of incineration. There were a lot of cows, so I assume it would be a crude way.
 
And of course there was probably the release of dioxins.
What im more interested in is why the proteins get folded wrong in teh first place. One hypotehsis that gets no publicity now is that its due to manganese taking the place of copper in the protein when it is being formed.
 
Hypothetically we could stop a prion induce disease like Infectious Spongiform Encephalopathy by making a drug with a molecular conformation that will bind to the specific confirmation of the prion, thus jamming the prion from aggregating or preventing it from inducing the conformation change in its substrate type. Unfortunately for this to work the disease will have to be detected first which means much irreversible neurological damage has already been done, unless this drug can be made cheaply to disperse as a preventative measure (very unlikely).

prions.jpg

See those beta sheets on the prion? A drug could be made to bind to that specific formation, jamming of the sheets would hypothetically prevent the prion from arrogating (the prions stick to each other on these sheets and form long crystalline rods of protein that can not be destroyed by normal protein recycling pathways and eventually grow long enough to rupture the cell membrane) also it is theorized that these beta sheets directly convert the normal protein forms, jamming the sheets would prevent conversion.

Here’s a good question what is the proper pronunciation of prion: “prE-on” or “pI-ron” or “PrI-on”? I have heard all 3 use before.
 
I think it is the first one, "prE-on", but that's just what the professors at my school use. Go with what you find comfortable.

BTW, thanks for the info WellCooked (although I didn't ask the original question).
 
You mean I did not answer the original question by god your right, I been bad… tell me I have been naughty?
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Well begin a protein it is not destroyed by ionizing radiation and chemicals as easily. it also can survive everything but total vaporization combustion, as there are trillions of these proteins in a infected animal even after burning some of the proteins survive in the cinders by random luck of not being combusted.
 
Ive always heard Pri-on, in the UK. or pry-on might be better. Then as for it infecting mice, it might be that having the misfolded protein in your brain causes many of the newly crated ones to also be misfolded. Not that im a biochemist.
 
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