I have a question about Prions and what they do in the human body and there link to mad cow disease?

Question

1. What are prions?

2.What function do they have on the human body?

3.What's there conection with mad cow disease?

I know that mad cow disease is a Zoonotic disease like H.I.V..

Answer 1

1. Prions are very tiny infectious agents, and not much is known about them.

2. In mad cow disease (which is spread through prions), prions infect the brain, and the brain deteriorates.

3. Check out wikipedia: http://en.wikipedia.org/wiki/Bovine_spon...

Answer 2

1. Prions are just small bits of protein without any DNA/RNA.

2. They cause a neurodegenerative disease called kuru in humans.

3. Currently the most accepted theory is that the causative agent of mad cow disease is a modified form of a normal cell surface protein called a prion protein.

Prions are normal constituents of the body when in their normal form or conformation, but they can become twisted in a conformational change ( a change in shape - in the way the molecule is folded), and then they are thought to cause disease.

Answer 3

Here is an article about prions from the Centers for Disease Control:

http://www.cdc.gov/ncidod/dvrd/prions/

And another from the Genetic Science Learning Center at the University of Utah:

http://learn.genetics.utah.edu/features/...

And one from a microbiology web site:

http://www.microbiologybytes.com/virolog...

Prions are proteins - chains of amino acids.  In fact, the word "prion" is an acronym for PROteinaceous INfectious particle.  But here's what makes them so mysterious and so confusing and so difficult to kill:  They're not alive.  A bacterium or a fungus is a cellular organism, and they're alive.  A prion doesn't have a cellular structure.  They CAN reproduce on their own, and they can become infectious.

Prions exist in the human body in various cell types but are primarily found in the brain.  Scientists think that they play a role in communication between neurons as well as in cell death and in sleep patterns.  The disease-causing prions actually have a different shape than their "normal" counterparts.  The abnormal prions stick to each other and eventually make long protein chains, called "amyloid fibers".  It's these fibers that cause the damage in the brain that eventually kills the patient.

Mad cow disease is just one of the diseases believed to be caused by prions.  In all these diseases, the brain tissue is gradually destroyed.  The prions destroy brain cells in little clusters so that eventually the brain looks like it has holes all through it, like a sponge.  That's why you'll hear the word "spongiform" in conjunction with these diseases (mad cow disease is BSE - bovine spongiform encephalopathy).