Fiche publication
Date publication
février 2004
Journal
The American journal of pathology
Auteurs
Membres identifiés du Cancéropôle Est :
Pr CHAMBON Pierre
,
Dr METZGER Daniel
Tous les auteurs :
Mori M, Metzger D, Picaud S, Hindelang C, Simonutti M, Sahel J, Chambon P, Mark M
Lien Pubmed
Résumé
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 alpha 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 alpha (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 alpha (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 alpha 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 alpha signaling pathways in the RPE in human retinal diseases.
Mots clés
Animals, Carrier Proteins, biosynthesis, Electroretinography, Eye, embryology, Eye Proteins, biosynthesis, Immunohistochemistry, In Situ Nick-End Labeling, Mice, Mice, Mutant Strains, Mice, Transgenic, Photoreceptor Cells, pathology, Pigment Epithelium of Eye, pathology, Protein Biosynthesis, Proteins, Receptors, G-Protein-Coupled, biosynthesis, Receptors, Retinoic Acid, deficiency, Retinal Diseases, etiology, Retinoid X Receptors, Reverse Transcriptase Polymerase Chain Reaction, Transcription Factors, deficiency, cis-trans-Isomerases
Référence
Am. J. Pathol.. 2004 Feb;164(2):701-10
