OVERVIEW of Protein misfolding diseases

Every protein's function is determined by its shape: the precise positioning of its various chemical components gives a protein the biophysical properties that make it a reliable link in a crucial biological process. If the protein becomes misfolded, it will typically lose its normal biological function and can take on new qualities that are pathological.

Crystallins, the predominant structural proteins of the eye’s natural lens, are susceptible to the accumulation of damage and stress related modifications over the course of a person’s life. This kind of damage is normally irreversible because the lens cells lack the ability to degrade proteins and re-synthesize new copies. Eventually, with aging, these modifications destabilize the proteins enough to cause aggregation.

Crystallin misfolding and aggregation changes the structural and optical properties of the protein, and the accumulation of aggregated crystallin causes the lens to increasingly lose its elasticity and transparency.