Researchers at the University of Alicante, Spain, have been studying the protective effects of a potential drug therapy, Tauroursodeoxicholic Acid (TUDCA), to prevent degeneration of the retina. Retinitis pigmentosa (RP) causes photoreceptor degeneration in the retina and eventually results in a global retinal remodeling phenomenon. Retinal remodeling can best be understood as reactive changes in the retina’s environment by different cells, as a result of photoreceptor damage. Oxygen metabolism in the retina becomes imbalanced as RP progresses, which increases oxidative stress in the retina, affecting other retinal cellular structures such as glial cells and retinal vasculature. TUDCA is a bile acid that has been shown to have neuroprotective effects and antioxidant properties. It is thought that this compound can decrease the oxidative stress in the retina, repair blood vessels in the surrounding areas, and decrease the expression of other inflammatory markers. In their study, it was found that rats with RP treated with TUDCA displayed better-preserved vessels, partial photoreceptor preservation, and decreased retinal remodeling effect in the retina.
What this means for Usher syndrome: If this compound is able to mitigate the effects of end-stage retinal degeneration, then it is hopeful that functional vision may be preserved in humans with later-stages of RP.
