Mitochondrial Defects Drive Degenerative Retinal Diseases

Vitamin A Derivatives as Treatment Options for Retinal Degenerative Diseases

The visual cycle is a sequential enzymatic reaction for vitamin A, all-trans-retinol, occurring in the outer layer of the human retina and is essential for the maintenance of vision. The central source of retinol is derived from dietary intake of both retinol and pro-vitamin A carotenoids. A series of enzymatic reactions, located in both the photoreceptor outer segment and the retinal pigment e...

Contribution of Microglia-Mediated Neuroinflammation to Retinal Degenerative Diseases

Retinal degenerative diseases are major causes of vision loss and blindness worldwide and are characterized by chronic and progressive neuronal loss. One common feature of retinal degenerative diseases and brain neurodegenerative diseases is chronic neuroinflammation. There is growing evidence that retinal microglia, as in the brain, become activated in the course of retinal degenerative diseas...

Gene therapy to treat inherited and complex retinal degenerative diseases

These are exciting times in the field of retinal gene therapy, as impressive successes have been achieved by recombinant adenoassociated viral (rAAV) gene delivery of Rpe65 or Rep1 to the retinal pigment epithelium of patients with Lebers congential amaurosis or choroideremia, respectively.1–5 Instead of being left with incurable blindness, patients are reporting restoration of vision. An advan...

Mitochondrial genetics: a paradigm for aging and degenerative diseases?

Studies of diseases caused by mitochondrial DNA mutations suggest that a variety of degenerative processes may be associated with defects in oxidative phosphorylation (OXPHOS). Application of this hypothesis has provided new insights into such diverse clinical problems as ischemic heart disease, late-onset diabetes, Parkinson's disease, Alzheimer's disease, and aging.

Discharge patterns of retinal ganglion cells in rodent models of degenerative retinal diseases