Thursday, February 11, 2010

Describe the protein IAG2 causing the disease Scarpie; What real world applications does it have?












Protein IAG2


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I found an interest in the protein IAG2, commonly called
scrapie, which is a neuro-degenerative disease found in sheep. This is closely related "mad cow disease" as well as many other similar diseases including human infecting ones.


Structurally the infecting prion, which is mutant PrPsc (Prion protein) has several differences that set it apart from its non mutant form. This infecting form contains a large amount of Beta pleated sheets, unlike the non mutant form which contains a high number of Alpha helix. The mutant form also contains amyloid fibers, unlike the non mutant strain, this aids in it virulence. The most complex part of the change between them is that these mutant forms interact between protein molecules. This leads to the actual spongiform or plaques of the disease. These holes formed in the brain cause altered mental status, ataxia, convulsions, and death.


No Cure has been found to treat scrapie but scientist have made some progress with computer simulations, and injections with mice. This infection is passed by the digestion of an infected organism, or a bi product of the organism. It can also infect by something as simple as a cut.
(ncbi.nlm.nih.gov)




Here is a picture the non mutant to mutant form: