Abstract
The retinal pigment epithelium (RPE) performs several essential functions in the retina. It is closely linked with the photoreceptors and in particular with the external segment of the photoreceptors, for which it ensures daily renewal by phagocytosis as well as their protection against phototoxicity. The RPE also provides transepithelial transport of ions and nutrients to the photoreceptors, and of water to the choroid. The RPE also regenerates the visual pigment that takes place in the RPE and in the photoreceptors. In addition, the EPR will also secrete cytokines and growth factors which will be essential for the survival and growth of the photoreceptors and the choroid. One of the most frequent causes of retinal blindness is genetic and linked to mutations in the genes expressed in the photoreceptors and/or in the RPE. As is the case for choroideremia which is a retinal dystrophy characterized by a progressive loss of photoreceptors and RPE. However, the absence of an appropriate animal model compromises the implementation of studies to elucidate the pathophysiological bases of this disease and therefore the arrival at a clinical stage of trials of new therapies. In order to overcome this, a model of RPE derived from patient stem cells (iPSC) was generated in order to deepen the pathophysiological studies on choroideremia in RPE derived from iPSC cells, to better understand this pathology and to evaluate the effectiveness of a treatment.