Prions and Mad Cow Disease

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Once the normal form is Pietro Faccioli, a physicist from the same university and affiliated to the Italian National Institute of Nuclear Physics.

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Prions are unusual infectious agents made by aberrantly folded forms of a physiological protein called the cellular prion protein, or PrPC. These pathogens are known to replicate in absence of genetic material by recruiting normal PrPC molecules at the surface of cells and forcing them to change conformation and become infectious themselves.

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However, such information is instrumental to rationally design drugs against these agents. In an attempt to fill this gap, we found unexpected help from a discipline usually considered far away from biology or chemistry, that is particles physics. In the end there is so much of the defective structure that small accumulations build up in the cells which then die.

Researchers tested different blends of LCP on mice infected with prions. Mice that got the LCP molecules before they were infected survived for days, while those that were injected with molecules after the time of infection survived for days. The findings were published in Science Translational Medicine.

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Previously it was not possible to control in detail what the different molecules would be like, but now we can. The researchers now hope to find a molecule that also affects these diseases in order to get closer to a cure for them in the future too.

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Images Above right: Brain tissue from a prion infected mouse shows that nerve cells blue died and were replaced by empty cavities. Above, right: In the same area of the brain, in tissue from a prion infected mouse that has been treated with the molecule, no changes can be seen.

Newly discovered infectious prion structure shines light on mad cow disease

Photo: Vibeke Mathiesen Publication Structure-based drug design identifies polythiophenes as antiprion compounds Uli S. Herrmann, Anne K. Meier, K. Peter R.

CJD Creutzfeldt-Jakob Disease - Mayo Clinic