This specialized research center with more than 2,000 patients on file with retinitis pigmentosa and allied retinal degenerations proposes to continue studies on the course of these diseases as monitored with psychophysical and electroretinographic testing. Research is also planned to study postmortem donor eyes with these diseases with morphometric and biochemical analyses as well as tissue culture techniques to help define pathogenetic mechanisms. Attention will be given to evaluating in postmortem donor eyes those abnormalities known or thought to play a role in the pathogenesis of photoreceptor cell death in animal models of retinal degenerations. Data derived from clinical and laboratory studies will be evaluated with biostatistical methods to better define the course, region-specific changes in donor eyes, and possible effect modifiers. The hope is that this research will reveal pathogenetic mechanisms and thereby increase understanding of this group of diseases which affect an estimated 50,000 to 100,000 people in this country and countless others around the world.
