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(American Journal of Pathology. 2004;164:701-710.)
© 2004 American Society for Investigative Pathology

Retinal Dystrophy Resulting from Ablation of RXR in the Mouse Retinal Pigment Epithelium

Mikiro Mori^*, Daniel Metzger^*, Serge Picaud, Colette Hindelang^*, Manuel Simonutti, José Sahel, Pierre Chambon^* and Manuel Mark^*

From the Institut de Génétique et de Biologie Moléculaire et Cellulaire^* and the Laboratoire de Physiopathologie Cellulaire et Moléculaire de la Rétine, Centre National de la Recherche Scientifique, Institut National de la Santé et de la Recherche Médicale, Université Louis Pasteur, Collège de France, Strasbourg; and the Institut Clinique de la Souris, Strasbourg, France

Vitamin A (retinol) actions in eye development are mediated by retinoic acid receptors (RARs and RXRs). Using the Cre/loxP system, we have selectively ablated RXR in the retinal pigment epithelium (RPE), a cell monolayer critically involved in visual retinoid renewal and phagocytosis of photoreceptor outer segments. In the mutant (RXR ^rpe-/-) mice, RPE cells are morphologically and functionally abnormal and display decreased expression of proteins involved in the visual retinoid cycle, namely RPE65, CRALBP, and RGR. RXR ^rpe-/- mice also show alterations of photoreceptor cells including: 1) decrease in their number; 2) outer segment shortening and disorganization, and 3) reduced light responses in electroretinograms. These results indicate that RXR is required for normal maturation of the RPE, which is known to play essential roles in photoreceptor cell function and survival, and point to a possible involvement of RXR signaling pathways in the RPE in human retinal diseases.

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