Mad Cow Disease
A study in prions
Prions are abnormal forms of proteins in the brain that can be caused by infection or genetic mutation. Once a prion enters the brain, they begin to multiply and influence otherwise harmless (benign) proteins to misfold into their abnormal structure.
Normally, these proteins would be made up of flexible coils known as alpha helices. However, these coils become flattened into new structures called beta strands in prion proteins. This shape prevents cellular enzymes or “proteases” from modifying them. As a result, the proteases cannot break down the prions when the multiply. Instead, the prions will continue to increase inside of the brain, destroying the neurons they inhabit. In other words, prion diseases are progressive in nature, worsening over time.
This kind of neuron destruction eats apart brain tissue, filling it with holes like a sponge. Fittingly, this effect is called spongiform.
In fact, the characteristic spongiform patterns allow scientists to verify the presence of prion proteins once an infected organism has died. This is done by analyzing the organism’s brain tissue under a microscope. Scientists do not have accurate test for suspected prion diseases in living organisms.
Mad Cow Disease is one type of prion induced disease. It’s also known as BSE, or “bovine spongiform…
