Abstract
With increasing human longevity, protein unfolding and aggregation diseases are among the greatest threats to the health of aging humans and to the health care system. Protein unfolding and aggregation diseases underlie the most prominent causes of deteriorating visual function, neurodegeneration and cardiomyopathy with age. Progress on the development and screening of novel therapeutics for unfolding and aggregation diseases is limited by the difficulties of studying the mechanisms of protein unfolding and aggregation in vivo. We report on novel methods for the overexpression of proteins responsible for aggregation diseases in transparent lens cells where protein unfolding and aggregation can be evaluated and quantified using innovative, noninvasive optical methods including slit lamp biomicroscopy, confocal microscopy and dynamic laser scattering spectroscopy (Seeberger et al, (2004) J. of Biomed Optics. 9: 116–120; Greiling & Clark, (2008) Seminars in Cell & Developmental Biology 19:94–99; Muchowski et al (2008) J Biol Chem 283:6330).