Protein Folding, Misfolding and Neurodegenerative Disease: How Proteins Maintain Their Right Shapes and What Happens When They Don't.

Proteins control our lives down to the smallest detail. Even so, the question of how a protein is formed is one of life's great mysteries. In a split second, the floppy protein chain forms itself into a ball with a unique shape and function. Occasionally, however, they get trapped in a wrong shape and run amok with devastating consequences for the cells. The understanding of these protein folding and misfolding processes are critical for finding rational treatment of many debilitating conditions like Alzheimer's disease, ALS and the prion diseases. Basically, the underlying principle is simple: the tension between fat and water. Just as fat is attracted to fat, and water to water, the proteins are controlled in the cells and join together correctly to form the right shape all by themselves. If one part loosens, it is automatically pulled back. This remarkable ability to self assemble is at least partly orchestrated by amino acids that sit like guards making sure that no wrong knots are made. If you remove them, the proteins distort, stick together by exposure of their greasy interior and kill the cells. Suddenly, the uniting force has been turned against us. But the most fascinating thing about proteins is not that they can go wrong, but that they function at all. What stops chaos from taking over?